A 54-year-old man presented at our facility with a 3-month history of exertional breathlessness and purple blotches around his eyes. Examination revealed bilateral periorbital and perioral ecchymosis, purpuric spots along his waist, and waxy papules on his eyelids (FIGURE 1). In addition, the patient had macroglossia with nodular infiltration and irregular indentations at the lateral margin of his tongue (FIGURE 2).
The patient also had a raised jugular venous pressure and prominent atrial and ventricular waves. Further examination revealed a fourth heart sound over the left ventricular apex, as well as bilateral basal rales. All other systems were normal except for mild hepatomegaly.
Routine hematologic and biochemical lab work was unremarkable. X-rays of the spine and skull were normal, but a chest x-ray showed mild cardiomegaly. An electrocardiogram (EKG) showed a QS complex from leads V1 to V4 (a pseudo-infarction pattern; FIGURE 3A). An echocardiogram showed biatrial enlargement, left ventricular hypertrophy with a left ventricular ejection fraction of 48%, a speckled pattern on the myocardium, a thickened interatrial septum, and mild pericardial effusion (FIGURE 3B).
A color Doppler revealed mild mitral and tricuspid regurgitation with a restrictive pattern of mitral valve flow. Serum protein electrophoresis was normal.
WHAT IS YOUR DIAGNOSIS? HOW WOULD YOU TREAT THIS PATIENT?
Diagnosis: Primary systemic amyloidosis
A diagnosis of primary systemic amyloidosis was confirmed with histopathologic examination of the abdominal fat pad using Congo red stain. Clinical, imaging, and laboratory features supported this diagnosis.
Primary systemic amyloidosis (also known as light-chain amyloidosis) is the most common type of systemic amyloidosis, affecting an estimated 5 to 12 million people per year.1,2 It occurs when there is a buildup of the abnormal protein amyloid. Organs that may be affected include the heart, kidneys, skin, nerves, and liver. There are no clear environmental, racial, or genetic risk factors for this condition.
With primary systemic amyloidosis, the ecchymosis present around the eyes may also appear elsewhere on the body (pinch purpura). Other symptoms may include macroglossia; sensory and autonomic neuropathy; and concomitant renal, cardiac, and hepatic involvement. In elderly patients with these symptoms, myeloma-associated systemic amyloidosis should be ruled out.2 Histopathologic examination of the abdominal fat pad or rectum is usually diagnostic.
Systemic amyloidosis and the heart
In patients with symptoms of congestive heart failure, a finding of thick heart walls on echocardiogram may indicate cardiac amyloidosis, particularly if there is no other underlying heart disease that could explain such findings. An even stronger indicator is the additional finding of low-voltage complexes on EKG.3
Periorbital ecchymosis can be a sign of many conditions
Bilateral periorbital ecchymosis, also known as “raccoon eyes,” was an important clinical clue to the diagnosis in our patient, but multiple conditions should be considered when raccoon eyes are present.
Basal skull fractureoccurs with a history of trauma. Clinical and radiologic signs of injuries can usually be found in other areas of the body.6
Periorbital cellulitis presents with unilateral erythematous periorbital swelling. A rapid increase in the patient’s temperature and swelling of tissue may occur. Movement of the extraocular muscles and visual acuity are usually normal.7
Blood dyscrasias usually involve a history of external bleeding.7 A thorough laboratory evaluation, including a complete blood count, platelet function tests, and a blood coagulation profile, is usually sufficient to exclude these cases.
A variety of treatment options
Clinicians have used angiotensin-converting enzyme inhibitors, long-acting nitrates, vasodilators, and diuretics to treat cardiac amyloidosis with varying results. For patients with atrial fibrillation (AF), ibutilide and amiodarone are useful antiarrhythmic drugs.3,8 In addition, experts recommend anticoagulation therapy with warfarin, dabigatran, or rivaroxaban for patients with AF because of the high risk of stroke.3,8 Symptomatic bradycardia and high-grade conduction-system disease usually require pacemaker implantation.
A guarded prognosis.The prognosis for patients with primary systemic amyloidosis is usually poor. Cardiac failure and renal failure are the major causes of death. The median survival time is 13 months, and only 5% of patients survive longer than 10 years.4,5
Our patientwas prescribed furosemide 40 mg/d, ramipril 1.25 mg/d, and spironolactone 25 mg/d. Within a couple weeks, his symptoms improved. However, 3 months after being diagnosed, the patient succumbed to heart failure.
CORRESPONDENCE Sudip Kumar Ghosh, MD, DNB, Department of Dermatology, Venereology, and Leprosy, R. G. Kar Medical College, 1, Khudiram Bose Sarani, Kolkata, West Bengal 700004, India; [email protected].
References
1. Gertz MA. The classification and typing of amyloid deposits. Am J Clin Pathol. 2004;121:787-789.
2. Sanchorawala V. Light-chain (AL) amyloidosis: diagnosis and treatment. Clin J Am Soc Nephrol. 2006;1:1331-1341.
4. Kyle RA, Gertz MA, Greipp PR, et al. A trial of three regimens for primary amyloidosis: colchicine alone, melphalan and prednisone, and melphalan, prednisone, and colchicine. N Engl J Med. 1997;336:1202-1207.
5. Kyle RA, Gertz MA, Greipp PR, et al. Long-term survival (10 years or more) in 30 patients with primary amyloidosis. Blood. 1999;93:1062-1066.
6. Somasundaram A, Laxton AW, Perrin RG. The clinical features of periorbital ecchymosis in a series of trauma patients. Injury. 2014;45:203-205.
7. Ghosh SK, Dutta A, Basu M. Raccoon eyes in a case of metastatic neuroblastoma. Indian J Dermatol Venereol Leprol. 2012;78:740-741.
8. Hassan W, Al-Sergani H, Mourad W, et al. Amyloid heart disease. New frontiers and insights in pathophysiology, diagnosis, and management. Tex Heart Inst J. 2005;32:178-184.
Department of Dermatology, Venereology, and Leprosy (Dr. Sudip Kumar Ghosh), Department of Cardiology (Drs. Majumder and Sandip Ghosh), and the Department of Surgery (Dr. Das), R. G. Kar Medical College and Hospital, West Bengal, India; Department of Biochemistry, College of Medicine & Sagore Dutta Hospital, West Bengal, India (Dr. Chatterjee) [email protected]
DEPARTMENT EDITOR Richard P. Usatine, MD University of Texas Health Science Center at San Antonio
The authors reported no potential conflict of interest relevant to this article.
Department of Dermatology, Venereology, and Leprosy (Dr. Sudip Kumar Ghosh), Department of Cardiology (Drs. Majumder and Sandip Ghosh), and the Department of Surgery (Dr. Das), R. G. Kar Medical College and Hospital, West Bengal, India; Department of Biochemistry, College of Medicine & Sagore Dutta Hospital, West Bengal, India (Dr. Chatterjee) [email protected]
DEPARTMENT EDITOR Richard P. Usatine, MD University of Texas Health Science Center at San Antonio
The authors reported no potential conflict of interest relevant to this article.
Author and Disclosure Information
Department of Dermatology, Venereology, and Leprosy (Dr. Sudip Kumar Ghosh), Department of Cardiology (Drs. Majumder and Sandip Ghosh), and the Department of Surgery (Dr. Das), R. G. Kar Medical College and Hospital, West Bengal, India; Department of Biochemistry, College of Medicine & Sagore Dutta Hospital, West Bengal, India (Dr. Chatterjee) [email protected]
DEPARTMENT EDITOR Richard P. Usatine, MD University of Texas Health Science Center at San Antonio
The authors reported no potential conflict of interest relevant to this article.
A 54-year-old man presented at our facility with a 3-month history of exertional breathlessness and purple blotches around his eyes. Examination revealed bilateral periorbital and perioral ecchymosis, purpuric spots along his waist, and waxy papules on his eyelids (FIGURE 1). In addition, the patient had macroglossia with nodular infiltration and irregular indentations at the lateral margin of his tongue (FIGURE 2).
The patient also had a raised jugular venous pressure and prominent atrial and ventricular waves. Further examination revealed a fourth heart sound over the left ventricular apex, as well as bilateral basal rales. All other systems were normal except for mild hepatomegaly.
Routine hematologic and biochemical lab work was unremarkable. X-rays of the spine and skull were normal, but a chest x-ray showed mild cardiomegaly. An electrocardiogram (EKG) showed a QS complex from leads V1 to V4 (a pseudo-infarction pattern; FIGURE 3A). An echocardiogram showed biatrial enlargement, left ventricular hypertrophy with a left ventricular ejection fraction of 48%, a speckled pattern on the myocardium, a thickened interatrial septum, and mild pericardial effusion (FIGURE 3B).
A color Doppler revealed mild mitral and tricuspid regurgitation with a restrictive pattern of mitral valve flow. Serum protein electrophoresis was normal.
WHAT IS YOUR DIAGNOSIS? HOW WOULD YOU TREAT THIS PATIENT?
Diagnosis: Primary systemic amyloidosis
A diagnosis of primary systemic amyloidosis was confirmed with histopathologic examination of the abdominal fat pad using Congo red stain. Clinical, imaging, and laboratory features supported this diagnosis.
Primary systemic amyloidosis (also known as light-chain amyloidosis) is the most common type of systemic amyloidosis, affecting an estimated 5 to 12 million people per year.1,2 It occurs when there is a buildup of the abnormal protein amyloid. Organs that may be affected include the heart, kidneys, skin, nerves, and liver. There are no clear environmental, racial, or genetic risk factors for this condition.
With primary systemic amyloidosis, the ecchymosis present around the eyes may also appear elsewhere on the body (pinch purpura). Other symptoms may include macroglossia; sensory and autonomic neuropathy; and concomitant renal, cardiac, and hepatic involvement. In elderly patients with these symptoms, myeloma-associated systemic amyloidosis should be ruled out.2 Histopathologic examination of the abdominal fat pad or rectum is usually diagnostic.
Systemic amyloidosis and the heart
In patients with symptoms of congestive heart failure, a finding of thick heart walls on echocardiogram may indicate cardiac amyloidosis, particularly if there is no other underlying heart disease that could explain such findings. An even stronger indicator is the additional finding of low-voltage complexes on EKG.3
Periorbital ecchymosis can be a sign of many conditions
Bilateral periorbital ecchymosis, also known as “raccoon eyes,” was an important clinical clue to the diagnosis in our patient, but multiple conditions should be considered when raccoon eyes are present.
Basal skull fractureoccurs with a history of trauma. Clinical and radiologic signs of injuries can usually be found in other areas of the body.6
Periorbital cellulitis presents with unilateral erythematous periorbital swelling. A rapid increase in the patient’s temperature and swelling of tissue may occur. Movement of the extraocular muscles and visual acuity are usually normal.7
Blood dyscrasias usually involve a history of external bleeding.7 A thorough laboratory evaluation, including a complete blood count, platelet function tests, and a blood coagulation profile, is usually sufficient to exclude these cases.
A variety of treatment options
Clinicians have used angiotensin-converting enzyme inhibitors, long-acting nitrates, vasodilators, and diuretics to treat cardiac amyloidosis with varying results. For patients with atrial fibrillation (AF), ibutilide and amiodarone are useful antiarrhythmic drugs.3,8 In addition, experts recommend anticoagulation therapy with warfarin, dabigatran, or rivaroxaban for patients with AF because of the high risk of stroke.3,8 Symptomatic bradycardia and high-grade conduction-system disease usually require pacemaker implantation.
A guarded prognosis.The prognosis for patients with primary systemic amyloidosis is usually poor. Cardiac failure and renal failure are the major causes of death. The median survival time is 13 months, and only 5% of patients survive longer than 10 years.4,5
Our patientwas prescribed furosemide 40 mg/d, ramipril 1.25 mg/d, and spironolactone 25 mg/d. Within a couple weeks, his symptoms improved. However, 3 months after being diagnosed, the patient succumbed to heart failure.
CORRESPONDENCE Sudip Kumar Ghosh, MD, DNB, Department of Dermatology, Venereology, and Leprosy, R. G. Kar Medical College, 1, Khudiram Bose Sarani, Kolkata, West Bengal 700004, India; [email protected].
A 54-year-old man presented at our facility with a 3-month history of exertional breathlessness and purple blotches around his eyes. Examination revealed bilateral periorbital and perioral ecchymosis, purpuric spots along his waist, and waxy papules on his eyelids (FIGURE 1). In addition, the patient had macroglossia with nodular infiltration and irregular indentations at the lateral margin of his tongue (FIGURE 2).
The patient also had a raised jugular venous pressure and prominent atrial and ventricular waves. Further examination revealed a fourth heart sound over the left ventricular apex, as well as bilateral basal rales. All other systems were normal except for mild hepatomegaly.
Routine hematologic and biochemical lab work was unremarkable. X-rays of the spine and skull were normal, but a chest x-ray showed mild cardiomegaly. An electrocardiogram (EKG) showed a QS complex from leads V1 to V4 (a pseudo-infarction pattern; FIGURE 3A). An echocardiogram showed biatrial enlargement, left ventricular hypertrophy with a left ventricular ejection fraction of 48%, a speckled pattern on the myocardium, a thickened interatrial septum, and mild pericardial effusion (FIGURE 3B).
A color Doppler revealed mild mitral and tricuspid regurgitation with a restrictive pattern of mitral valve flow. Serum protein electrophoresis was normal.
WHAT IS YOUR DIAGNOSIS? HOW WOULD YOU TREAT THIS PATIENT?
Diagnosis: Primary systemic amyloidosis
A diagnosis of primary systemic amyloidosis was confirmed with histopathologic examination of the abdominal fat pad using Congo red stain. Clinical, imaging, and laboratory features supported this diagnosis.
Primary systemic amyloidosis (also known as light-chain amyloidosis) is the most common type of systemic amyloidosis, affecting an estimated 5 to 12 million people per year.1,2 It occurs when there is a buildup of the abnormal protein amyloid. Organs that may be affected include the heart, kidneys, skin, nerves, and liver. There are no clear environmental, racial, or genetic risk factors for this condition.
With primary systemic amyloidosis, the ecchymosis present around the eyes may also appear elsewhere on the body (pinch purpura). Other symptoms may include macroglossia; sensory and autonomic neuropathy; and concomitant renal, cardiac, and hepatic involvement. In elderly patients with these symptoms, myeloma-associated systemic amyloidosis should be ruled out.2 Histopathologic examination of the abdominal fat pad or rectum is usually diagnostic.
Systemic amyloidosis and the heart
In patients with symptoms of congestive heart failure, a finding of thick heart walls on echocardiogram may indicate cardiac amyloidosis, particularly if there is no other underlying heart disease that could explain such findings. An even stronger indicator is the additional finding of low-voltage complexes on EKG.3
Periorbital ecchymosis can be a sign of many conditions
Bilateral periorbital ecchymosis, also known as “raccoon eyes,” was an important clinical clue to the diagnosis in our patient, but multiple conditions should be considered when raccoon eyes are present.
Basal skull fractureoccurs with a history of trauma. Clinical and radiologic signs of injuries can usually be found in other areas of the body.6
Periorbital cellulitis presents with unilateral erythematous periorbital swelling. A rapid increase in the patient’s temperature and swelling of tissue may occur. Movement of the extraocular muscles and visual acuity are usually normal.7
Blood dyscrasias usually involve a history of external bleeding.7 A thorough laboratory evaluation, including a complete blood count, platelet function tests, and a blood coagulation profile, is usually sufficient to exclude these cases.
A variety of treatment options
Clinicians have used angiotensin-converting enzyme inhibitors, long-acting nitrates, vasodilators, and diuretics to treat cardiac amyloidosis with varying results. For patients with atrial fibrillation (AF), ibutilide and amiodarone are useful antiarrhythmic drugs.3,8 In addition, experts recommend anticoagulation therapy with warfarin, dabigatran, or rivaroxaban for patients with AF because of the high risk of stroke.3,8 Symptomatic bradycardia and high-grade conduction-system disease usually require pacemaker implantation.
A guarded prognosis.The prognosis for patients with primary systemic amyloidosis is usually poor. Cardiac failure and renal failure are the major causes of death. The median survival time is 13 months, and only 5% of patients survive longer than 10 years.4,5
Our patientwas prescribed furosemide 40 mg/d, ramipril 1.25 mg/d, and spironolactone 25 mg/d. Within a couple weeks, his symptoms improved. However, 3 months after being diagnosed, the patient succumbed to heart failure.
CORRESPONDENCE Sudip Kumar Ghosh, MD, DNB, Department of Dermatology, Venereology, and Leprosy, R. G. Kar Medical College, 1, Khudiram Bose Sarani, Kolkata, West Bengal 700004, India; [email protected].
References
1. Gertz MA. The classification and typing of amyloid deposits. Am J Clin Pathol. 2004;121:787-789.
2. Sanchorawala V. Light-chain (AL) amyloidosis: diagnosis and treatment. Clin J Am Soc Nephrol. 2006;1:1331-1341.
4. Kyle RA, Gertz MA, Greipp PR, et al. A trial of three regimens for primary amyloidosis: colchicine alone, melphalan and prednisone, and melphalan, prednisone, and colchicine. N Engl J Med. 1997;336:1202-1207.
5. Kyle RA, Gertz MA, Greipp PR, et al. Long-term survival (10 years or more) in 30 patients with primary amyloidosis. Blood. 1999;93:1062-1066.
6. Somasundaram A, Laxton AW, Perrin RG. The clinical features of periorbital ecchymosis in a series of trauma patients. Injury. 2014;45:203-205.
7. Ghosh SK, Dutta A, Basu M. Raccoon eyes in a case of metastatic neuroblastoma. Indian J Dermatol Venereol Leprol. 2012;78:740-741.
8. Hassan W, Al-Sergani H, Mourad W, et al. Amyloid heart disease. New frontiers and insights in pathophysiology, diagnosis, and management. Tex Heart Inst J. 2005;32:178-184.
References
1. Gertz MA. The classification and typing of amyloid deposits. Am J Clin Pathol. 2004;121:787-789.
2. Sanchorawala V. Light-chain (AL) amyloidosis: diagnosis and treatment. Clin J Am Soc Nephrol. 2006;1:1331-1341.
4. Kyle RA, Gertz MA, Greipp PR, et al. A trial of three regimens for primary amyloidosis: colchicine alone, melphalan and prednisone, and melphalan, prednisone, and colchicine. N Engl J Med. 1997;336:1202-1207.
5. Kyle RA, Gertz MA, Greipp PR, et al. Long-term survival (10 years or more) in 30 patients with primary amyloidosis. Blood. 1999;93:1062-1066.
6. Somasundaram A, Laxton AW, Perrin RG. The clinical features of periorbital ecchymosis in a series of trauma patients. Injury. 2014;45:203-205.
7. Ghosh SK, Dutta A, Basu M. Raccoon eyes in a case of metastatic neuroblastoma. Indian J Dermatol Venereol Leprol. 2012;78:740-741.
8. Hassan W, Al-Sergani H, Mourad W, et al. Amyloid heart disease. New frontiers and insights in pathophysiology, diagnosis, and management. Tex Heart Inst J. 2005;32:178-184.
Figure 1. Erythema of both hands.A 42-year-old businessman presented with recurrent redness, swelling, warmth, and burning pain on both hands and both feet for the preceding year (Figure 1). His symptoms were worse during heat exposure or physical effort and were relieved by immersing his hands and feet in cold water and by elevating his limbs. He had no other local or systemic symptoms, and he had not been on any medications. His medical history was noncontributory, and there was no family history of similar illness.
Figure 2. Blanching of erythema with pressure.The erythema blanched when pressure was applied (Figure 2). The affected areas were slightly tender. Other areas of his skin and mucosae were normal. Neurologic examination and examination of other systems were normal. Results of laboratory testing (complete blood cell count, biochemistry panel, antinuclear antibody test) and gastrointestinal endoscopy were normal.
Q: What is the diagnosis?
Fabry disease
Peripheral neuropathy
Polycythemia
Primary idiopathic erythromelalgia
Erythrodysesthesia syndrome
A: The correct diagnosis is primary idiopathic erythromelalgia.
Erythromelalgia is a relatively rare clinical condition of uncertain etiology, characterized by the triad of episodic redness, warmth, and burning pain in the extremities.1,2
The condition has primary (ie, no underlying cause is found) and secondary forms.1,3 The primary form may be inherited in an autosomal dominant manner (in which case the symptoms begin in childhood), or it may be idiopathic.4 On the other hand, erythromelalgia can also be secondary to polycythemia vera and other myeloproliferative disorders, connective tissue disorders, neuropathies, spinal cord diseases, carcinoma of the colon and thyroid, and astrocytomas.1–6
The common pathologic mechanism of erythromelalgia is thought to be microvascular arteriovenous shunting.5 A mutation in the voltage-gated sodium channel alpha subunit NaV1.7 may result in primary erythromelalgia. Small-fiber neuropathy2 can manifest as erythromelalgia and may respond to steroids.
The symptoms can be intermittent or, in rare cases, constant.3 The lower limbs are more commonly involved than the upper ones.3 Involvement is often bilateral and symmetric.3 The symptoms may worsen at night, after alcohol consumption, with higher environmental temperature, and with moderate exercise.3 In rare cases, ulceration and gangrene may occur. Patients may get relief by cooling the affected areas.2
DIAGNOSIS BY EXCLUSION OR BASED ON THE PRESENTATION
The diagnosis of erythromelalgia is based on a detailed history and physical examination during a painful episode.2,3 Because the condition is intermittent, only about two-thirds of patients have abnormal findings on physical examination at the time of presentation; in such cases, the diagnosis is based on the history alone.2
Testing is needed to exclude other diagnoses and to determine the cause of secondary erythromelalgia. Histopathologic study of lesions is not helpful, as the features are nonspecific and hence nondiagnostic.2
In our patient, the typical clinical features and the lack of an obvious cause on diagnostic testing confirmed the diagnosis.
THE DIFFERENTIAL DIAGNOSIS
Other conditions can be ruled out by clinical features and laboratory testing.
Fabry disease causes paresthesia and burning pain in the extremities but not erythema. Characteristic dark red keratotic papules are seen all over the body (angiokeratoma corporis diffusum). It is often associated with progressive renal insufficiency.
Peripheral neuropathy of varying causes may also cause pain in the extremities but not erythema. Neurologic examination and a nerve conduction velocity study can resolve the diagnostic problem. Clinical features and laboratory testing often help to pinpoint the cause of neuropathy.
Polycythemia may cause erythema in the hands, feet, and face and mucosal engorgement. Telangiectasia, petechiae, and cyanosis may also occur. Our patient’s normal complete blood cell count excluded this condition.
Erythrodysesthesia syndrome, typically caused by chemotherapeutic drugs, was not included in the differential diagnosis since our patient had taken no medications.
Other causes of palmar erythema to rule out, depending on the patient’s presentation, may include thyrotoxicosis, chronic febrile illness, leukemia, hepatic insufficiency, chronic alcoholism, and rheumatoid arthritis.
TREATMENT AND PROGNOSIS
There is no definitive therapy for erythromelalgia.4 Treatment is often difficult and needs a multidisciplinary approach. Simple measures such as cooling2 (eg, applying cold towels, immersion in cool water, walking on cold floors) or elevating the affected extremity often relieve symptoms. Patients should avoid precipitating factors such as warmth, dependency of extremities, exercise, tight footwear, and alcohol intake.
If there is an underlying disease, treating the disease may also alleviate the symptoms.7 Aspirin2,7 is the therapy of choice for erythromelalgia in patients with an underlying myeloproliferative disorder, and some authors have advocated it for all patients with erythromelalgia unless there is a contraindication.7
Other possible first-line treatments include the synthetic prostaglandin E1 analogue misoprostol (Cytotec) and prostacyclins. Gabapentin (Neurontin), serotonin reuptake inhibitors such as sertaline (Zoloft) and venlafaxine (Effexor), and intravenous nitroprusside (Nitro-press) are considered second-line drugs.7
Surgical sympathectomy8 has also been tried, with variable results.
Outcomes in patients with erythromelalgia
In a case series from Mayo Clinic,2 approximately equal numbers of patients with erythromelalgia became worse, stayed the same, or got better, and the disease resolved in 10% over a mean of 8.7 years.
THE OUTCOME IN OUR PATIENT
We advised our patient to avoid strenuous activity in a warm environment and to work in cooler areas as much as possible. We told him to wrap his affected extremities with cold towels during attacks, and we prescribed aspirin (650 mg/day) for 3 months. The treatment did not cure his condition, but his symptoms lessened within 2 months. We later referred him to a pain clinic.
References
Kalgaard OM, Seem E, Kvernebo K. Erythromelalgia: a clinical study of 87 cases. Intern Med1997; 242:191–197.
Davis MD, O’Fallon WM, Rogers RS, Rooke TW. Natural history of erythromelalgia: presentation and outcome in 168 patients. Arch Dermatol2000; 136:330–336.
Galimberti D, Pontón A, Rubio L, et al. A case of primary erythromelalgia. J Eur Acad Dermatol Venereol2009; 23:1338–1339.
Mørk C, Kvernebo K, Asker CL, Salerud EG. Reduced skin capillary density during attacks of erythromelalgia implies arteriovenous shunting as pathogenetic mechanism. J Invest Dermatol2002; 119:949–953.
James WD, Berger TG, Elston DM. Andrews’ Diseases of the Skin: Clinical Dermatology. 10th ed.Philadelphia, PA: Saunders Elsevier; 2006.
Mørk C, Kvernebo K. Erythromelalgia. In:Lebwohl MG, Heymann WR, Berth-Jones J, Koulson Y. editors. Treatment of Skin Disease: Comprehensive Therapeutic Strategies. 3rd ed.Philadelphia, PA: Saunders Elsevier; 2010:236–238.
Seishima M, Kanoh H, Izumi T, et al. A refractory case of secondary erythermalgia successfully treated with lumbar sympathetic ganglion block. Br J Dermatol2000; 143:868–872.
Sudip Kumar Ghosh, MD, DNB Assistant Professor, Department of Dermatology, Venereology, and Leprosy, R. G. Kar Medical College, Kolkata, India
Debabrata Bandyopadhyay, MD Professor, Department of Dermatology, Venereology, and Leprosy, Calcutta Medical College, Kolkata, India
Loknath Ghoshal, MD Resident Medical Officer-Cum-Clinical Tutor, Department of Dermatology, Venereology, and Leprosy, R. G. Kar Medical College, Kolkata, India
Address: Sudip Kumar Ghosh, MD, DNB, Department of Dermatology, Venereology, and Leprosy, R. G. Kar Medical College, 1, Khudiram Bose Road, Kolkata 700004, West Bengal, India; e-mail [email protected]
Sudip Kumar Ghosh, MD, DNB Assistant Professor, Department of Dermatology, Venereology, and Leprosy, R. G. Kar Medical College, Kolkata, India
Debabrata Bandyopadhyay, MD Professor, Department of Dermatology, Venereology, and Leprosy, Calcutta Medical College, Kolkata, India
Loknath Ghoshal, MD Resident Medical Officer-Cum-Clinical Tutor, Department of Dermatology, Venereology, and Leprosy, R. G. Kar Medical College, Kolkata, India
Address: Sudip Kumar Ghosh, MD, DNB, Department of Dermatology, Venereology, and Leprosy, R. G. Kar Medical College, 1, Khudiram Bose Road, Kolkata 700004, West Bengal, India; e-mail [email protected]
Author and Disclosure Information
Sudip Kumar Ghosh, MD, DNB Assistant Professor, Department of Dermatology, Venereology, and Leprosy, R. G. Kar Medical College, Kolkata, India
Debabrata Bandyopadhyay, MD Professor, Department of Dermatology, Venereology, and Leprosy, Calcutta Medical College, Kolkata, India
Loknath Ghoshal, MD Resident Medical Officer-Cum-Clinical Tutor, Department of Dermatology, Venereology, and Leprosy, R. G. Kar Medical College, Kolkata, India
Address: Sudip Kumar Ghosh, MD, DNB, Department of Dermatology, Venereology, and Leprosy, R. G. Kar Medical College, 1, Khudiram Bose Road, Kolkata 700004, West Bengal, India; e-mail [email protected]
Figure 1. Erythema of both hands.A 42-year-old businessman presented with recurrent redness, swelling, warmth, and burning pain on both hands and both feet for the preceding year (Figure 1). His symptoms were worse during heat exposure or physical effort and were relieved by immersing his hands and feet in cold water and by elevating his limbs. He had no other local or systemic symptoms, and he had not been on any medications. His medical history was noncontributory, and there was no family history of similar illness.
Figure 2. Blanching of erythema with pressure.The erythema blanched when pressure was applied (Figure 2). The affected areas were slightly tender. Other areas of his skin and mucosae were normal. Neurologic examination and examination of other systems were normal. Results of laboratory testing (complete blood cell count, biochemistry panel, antinuclear antibody test) and gastrointestinal endoscopy were normal.
Q: What is the diagnosis?
Fabry disease
Peripheral neuropathy
Polycythemia
Primary idiopathic erythromelalgia
Erythrodysesthesia syndrome
A: The correct diagnosis is primary idiopathic erythromelalgia.
Erythromelalgia is a relatively rare clinical condition of uncertain etiology, characterized by the triad of episodic redness, warmth, and burning pain in the extremities.1,2
The condition has primary (ie, no underlying cause is found) and secondary forms.1,3 The primary form may be inherited in an autosomal dominant manner (in which case the symptoms begin in childhood), or it may be idiopathic.4 On the other hand, erythromelalgia can also be secondary to polycythemia vera and other myeloproliferative disorders, connective tissue disorders, neuropathies, spinal cord diseases, carcinoma of the colon and thyroid, and astrocytomas.1–6
The common pathologic mechanism of erythromelalgia is thought to be microvascular arteriovenous shunting.5 A mutation in the voltage-gated sodium channel alpha subunit NaV1.7 may result in primary erythromelalgia. Small-fiber neuropathy2 can manifest as erythromelalgia and may respond to steroids.
The symptoms can be intermittent or, in rare cases, constant.3 The lower limbs are more commonly involved than the upper ones.3 Involvement is often bilateral and symmetric.3 The symptoms may worsen at night, after alcohol consumption, with higher environmental temperature, and with moderate exercise.3 In rare cases, ulceration and gangrene may occur. Patients may get relief by cooling the affected areas.2
DIAGNOSIS BY EXCLUSION OR BASED ON THE PRESENTATION
The diagnosis of erythromelalgia is based on a detailed history and physical examination during a painful episode.2,3 Because the condition is intermittent, only about two-thirds of patients have abnormal findings on physical examination at the time of presentation; in such cases, the diagnosis is based on the history alone.2
Testing is needed to exclude other diagnoses and to determine the cause of secondary erythromelalgia. Histopathologic study of lesions is not helpful, as the features are nonspecific and hence nondiagnostic.2
In our patient, the typical clinical features and the lack of an obvious cause on diagnostic testing confirmed the diagnosis.
THE DIFFERENTIAL DIAGNOSIS
Other conditions can be ruled out by clinical features and laboratory testing.
Fabry disease causes paresthesia and burning pain in the extremities but not erythema. Characteristic dark red keratotic papules are seen all over the body (angiokeratoma corporis diffusum). It is often associated with progressive renal insufficiency.
Peripheral neuropathy of varying causes may also cause pain in the extremities but not erythema. Neurologic examination and a nerve conduction velocity study can resolve the diagnostic problem. Clinical features and laboratory testing often help to pinpoint the cause of neuropathy.
Polycythemia may cause erythema in the hands, feet, and face and mucosal engorgement. Telangiectasia, petechiae, and cyanosis may also occur. Our patient’s normal complete blood cell count excluded this condition.
Erythrodysesthesia syndrome, typically caused by chemotherapeutic drugs, was not included in the differential diagnosis since our patient had taken no medications.
Other causes of palmar erythema to rule out, depending on the patient’s presentation, may include thyrotoxicosis, chronic febrile illness, leukemia, hepatic insufficiency, chronic alcoholism, and rheumatoid arthritis.
TREATMENT AND PROGNOSIS
There is no definitive therapy for erythromelalgia.4 Treatment is often difficult and needs a multidisciplinary approach. Simple measures such as cooling2 (eg, applying cold towels, immersion in cool water, walking on cold floors) or elevating the affected extremity often relieve symptoms. Patients should avoid precipitating factors such as warmth, dependency of extremities, exercise, tight footwear, and alcohol intake.
If there is an underlying disease, treating the disease may also alleviate the symptoms.7 Aspirin2,7 is the therapy of choice for erythromelalgia in patients with an underlying myeloproliferative disorder, and some authors have advocated it for all patients with erythromelalgia unless there is a contraindication.7
Other possible first-line treatments include the synthetic prostaglandin E1 analogue misoprostol (Cytotec) and prostacyclins. Gabapentin (Neurontin), serotonin reuptake inhibitors such as sertaline (Zoloft) and venlafaxine (Effexor), and intravenous nitroprusside (Nitro-press) are considered second-line drugs.7
Surgical sympathectomy8 has also been tried, with variable results.
Outcomes in patients with erythromelalgia
In a case series from Mayo Clinic,2 approximately equal numbers of patients with erythromelalgia became worse, stayed the same, or got better, and the disease resolved in 10% over a mean of 8.7 years.
THE OUTCOME IN OUR PATIENT
We advised our patient to avoid strenuous activity in a warm environment and to work in cooler areas as much as possible. We told him to wrap his affected extremities with cold towels during attacks, and we prescribed aspirin (650 mg/day) for 3 months. The treatment did not cure his condition, but his symptoms lessened within 2 months. We later referred him to a pain clinic.
Figure 1. Erythema of both hands.A 42-year-old businessman presented with recurrent redness, swelling, warmth, and burning pain on both hands and both feet for the preceding year (Figure 1). His symptoms were worse during heat exposure or physical effort and were relieved by immersing his hands and feet in cold water and by elevating his limbs. He had no other local or systemic symptoms, and he had not been on any medications. His medical history was noncontributory, and there was no family history of similar illness.
Figure 2. Blanching of erythema with pressure.The erythema blanched when pressure was applied (Figure 2). The affected areas were slightly tender. Other areas of his skin and mucosae were normal. Neurologic examination and examination of other systems were normal. Results of laboratory testing (complete blood cell count, biochemistry panel, antinuclear antibody test) and gastrointestinal endoscopy were normal.
Q: What is the diagnosis?
Fabry disease
Peripheral neuropathy
Polycythemia
Primary idiopathic erythromelalgia
Erythrodysesthesia syndrome
A: The correct diagnosis is primary idiopathic erythromelalgia.
Erythromelalgia is a relatively rare clinical condition of uncertain etiology, characterized by the triad of episodic redness, warmth, and burning pain in the extremities.1,2
The condition has primary (ie, no underlying cause is found) and secondary forms.1,3 The primary form may be inherited in an autosomal dominant manner (in which case the symptoms begin in childhood), or it may be idiopathic.4 On the other hand, erythromelalgia can also be secondary to polycythemia vera and other myeloproliferative disorders, connective tissue disorders, neuropathies, spinal cord diseases, carcinoma of the colon and thyroid, and astrocytomas.1–6
The common pathologic mechanism of erythromelalgia is thought to be microvascular arteriovenous shunting.5 A mutation in the voltage-gated sodium channel alpha subunit NaV1.7 may result in primary erythromelalgia. Small-fiber neuropathy2 can manifest as erythromelalgia and may respond to steroids.
The symptoms can be intermittent or, in rare cases, constant.3 The lower limbs are more commonly involved than the upper ones.3 Involvement is often bilateral and symmetric.3 The symptoms may worsen at night, after alcohol consumption, with higher environmental temperature, and with moderate exercise.3 In rare cases, ulceration and gangrene may occur. Patients may get relief by cooling the affected areas.2
DIAGNOSIS BY EXCLUSION OR BASED ON THE PRESENTATION
The diagnosis of erythromelalgia is based on a detailed history and physical examination during a painful episode.2,3 Because the condition is intermittent, only about two-thirds of patients have abnormal findings on physical examination at the time of presentation; in such cases, the diagnosis is based on the history alone.2
Testing is needed to exclude other diagnoses and to determine the cause of secondary erythromelalgia. Histopathologic study of lesions is not helpful, as the features are nonspecific and hence nondiagnostic.2
In our patient, the typical clinical features and the lack of an obvious cause on diagnostic testing confirmed the diagnosis.
THE DIFFERENTIAL DIAGNOSIS
Other conditions can be ruled out by clinical features and laboratory testing.
Fabry disease causes paresthesia and burning pain in the extremities but not erythema. Characteristic dark red keratotic papules are seen all over the body (angiokeratoma corporis diffusum). It is often associated with progressive renal insufficiency.
Peripheral neuropathy of varying causes may also cause pain in the extremities but not erythema. Neurologic examination and a nerve conduction velocity study can resolve the diagnostic problem. Clinical features and laboratory testing often help to pinpoint the cause of neuropathy.
Polycythemia may cause erythema in the hands, feet, and face and mucosal engorgement. Telangiectasia, petechiae, and cyanosis may also occur. Our patient’s normal complete blood cell count excluded this condition.
Erythrodysesthesia syndrome, typically caused by chemotherapeutic drugs, was not included in the differential diagnosis since our patient had taken no medications.
Other causes of palmar erythema to rule out, depending on the patient’s presentation, may include thyrotoxicosis, chronic febrile illness, leukemia, hepatic insufficiency, chronic alcoholism, and rheumatoid arthritis.
TREATMENT AND PROGNOSIS
There is no definitive therapy for erythromelalgia.4 Treatment is often difficult and needs a multidisciplinary approach. Simple measures such as cooling2 (eg, applying cold towels, immersion in cool water, walking on cold floors) or elevating the affected extremity often relieve symptoms. Patients should avoid precipitating factors such as warmth, dependency of extremities, exercise, tight footwear, and alcohol intake.
If there is an underlying disease, treating the disease may also alleviate the symptoms.7 Aspirin2,7 is the therapy of choice for erythromelalgia in patients with an underlying myeloproliferative disorder, and some authors have advocated it for all patients with erythromelalgia unless there is a contraindication.7
Other possible first-line treatments include the synthetic prostaglandin E1 analogue misoprostol (Cytotec) and prostacyclins. Gabapentin (Neurontin), serotonin reuptake inhibitors such as sertaline (Zoloft) and venlafaxine (Effexor), and intravenous nitroprusside (Nitro-press) are considered second-line drugs.7
Surgical sympathectomy8 has also been tried, with variable results.
Outcomes in patients with erythromelalgia
In a case series from Mayo Clinic,2 approximately equal numbers of patients with erythromelalgia became worse, stayed the same, or got better, and the disease resolved in 10% over a mean of 8.7 years.
THE OUTCOME IN OUR PATIENT
We advised our patient to avoid strenuous activity in a warm environment and to work in cooler areas as much as possible. We told him to wrap his affected extremities with cold towels during attacks, and we prescribed aspirin (650 mg/day) for 3 months. The treatment did not cure his condition, but his symptoms lessened within 2 months. We later referred him to a pain clinic.
References
Kalgaard OM, Seem E, Kvernebo K. Erythromelalgia: a clinical study of 87 cases. Intern Med1997; 242:191–197.
Davis MD, O’Fallon WM, Rogers RS, Rooke TW. Natural history of erythromelalgia: presentation and outcome in 168 patients. Arch Dermatol2000; 136:330–336.
Galimberti D, Pontón A, Rubio L, et al. A case of primary erythromelalgia. J Eur Acad Dermatol Venereol2009; 23:1338–1339.
Mørk C, Kvernebo K, Asker CL, Salerud EG. Reduced skin capillary density during attacks of erythromelalgia implies arteriovenous shunting as pathogenetic mechanism. J Invest Dermatol2002; 119:949–953.
James WD, Berger TG, Elston DM. Andrews’ Diseases of the Skin: Clinical Dermatology. 10th ed.Philadelphia, PA: Saunders Elsevier; 2006.
Mørk C, Kvernebo K. Erythromelalgia. In:Lebwohl MG, Heymann WR, Berth-Jones J, Koulson Y. editors. Treatment of Skin Disease: Comprehensive Therapeutic Strategies. 3rd ed.Philadelphia, PA: Saunders Elsevier; 2010:236–238.
Seishima M, Kanoh H, Izumi T, et al. A refractory case of secondary erythermalgia successfully treated with lumbar sympathetic ganglion block. Br J Dermatol2000; 143:868–872.
References
Kalgaard OM, Seem E, Kvernebo K. Erythromelalgia: a clinical study of 87 cases. Intern Med1997; 242:191–197.
Davis MD, O’Fallon WM, Rogers RS, Rooke TW. Natural history of erythromelalgia: presentation and outcome in 168 patients. Arch Dermatol2000; 136:330–336.
Galimberti D, Pontón A, Rubio L, et al. A case of primary erythromelalgia. J Eur Acad Dermatol Venereol2009; 23:1338–1339.
Mørk C, Kvernebo K, Asker CL, Salerud EG. Reduced skin capillary density during attacks of erythromelalgia implies arteriovenous shunting as pathogenetic mechanism. J Invest Dermatol2002; 119:949–953.
James WD, Berger TG, Elston DM. Andrews’ Diseases of the Skin: Clinical Dermatology. 10th ed.Philadelphia, PA: Saunders Elsevier; 2006.
Mørk C, Kvernebo K. Erythromelalgia. In:Lebwohl MG, Heymann WR, Berth-Jones J, Koulson Y. editors. Treatment of Skin Disease: Comprehensive Therapeutic Strategies. 3rd ed.Philadelphia, PA: Saunders Elsevier; 2010:236–238.
Seishima M, Kanoh H, Izumi T, et al. A refractory case of secondary erythermalgia successfully treated with lumbar sympathetic ganglion block. Br J Dermatol2000; 143:868–872.
A 12-year-old boy presents with painless swelling and ulceration on and around his nose that has progressed gradually over the last 6 months. The lesion has increased in size despite treatment with topical neomycin and oral erythromycin. He has no systemic symptoms.
Figure 1. Ulcerated plaque with destruction of the right nasal wing.On examination (Figure 1), we note an indurated, nontender plaque with scarring at places on his right cheek, nose, and the vermilion border of the lip. In addition, there are two purulent ulcerations on the nose partly destroying the right nasal wing. The upper lip is also infiltrated, studded with a solitary ulceration. There is no regional lymphadenopathy. An examination of systems is normal.
Cutaneous tuberculosis occurs in many forms, and lupus vulgaris is one of the most common.1 Lupus vulgaris usually arises as a result of hematogenous spread from an endogenous source. It may also arise from exogenous inoculation or as a complication of vaccination with bacille Calmette-Guérin.2
Several morphologic variants have been described.1,2 One form is characterized by plaques, often studded with psoriasiform scales. Large plaques may show irregular areas of scarring with islands of active lupus tissue and a thickened and hyperkeratotic margin. Ulcerative and mutilating variants of lupus vulgaris are characterized by scarring, ulceration, crusts over areas of necrosis, and destruction of the deep tissues and cartilage, resulting in deformities. The vegetative form produces marked infiltration, ulceration, and necrosis, with minimal scarring. Mucous membranes and cartilages are often destroyed. Tumor-like hypertrophic lesions and multiple papular and nodular lesions may also be seen. Nasal lesions may start as nodules, which may bleed and then ulcerate, sometimes resulting in cartilage destruction.
CLINICAL FEATURES AND LABORATORY WORKUP CLINCHED THE DIAGNOSIS
A number of factors helped to confirm the diagnosis in this patient:
A strongly positive Mantoux test (22-mm induration at 48 hours)
Acid-fast bacilli on Ziehl-Neelsen staining of the smear taken from the purulent ulceration
Isolation of Mycobacterium tuberculosis from the purulent exudates via culture in Lowenstein-Jensen medium
Figure 2. Epithelioid cell granuloma and giant cells (hematoxylin and eosin, × 100).A suggestive histopathologic picture (Figure 2)
The features on presentation
A significant clinical improvement within 2 months of starting antituberculosis therapy.
DIFFERENTIAL DIAGNOSIS
The differential diagnosis includes all the conditions in the question above. However, the absence of respiratory and renal involvement helps rule out Wegener granulomatosis; the absence of impaired sensation and nerve thickening helps rule out Hansen disease; and the absence of a nasal septal defect helps rule out Wegener granulomatosis, midline lethal granuloma, and Hansen disease.
On the other hand, the lupoid form of cutaneous leishmaniasis usually presents as an erythematous, infiltrated plaque that often closely resembles lupus vulgaris, but these lesions are usually less destructive than lupus vulgaris. However, the laboratory workup including the microbiological and histopathologic examination clearly excluded the other potential diagnoses in this patient.
TREATMENT
Lupus vulgaris is treated with standard antituberculosis therapy.3 The first phase of a fourdrug regimen is given for 2 months—isoniazid, rifampin (Rifadin), pyrazinamide, and ethambutol (Myambutol). The second phase consists of isoniazid and rifampin for 4 months.3
Early recognition and confirmation of the diagnosis followed by treatment are of immense importance for preventing permanent disfigurement.
References
Freitag DS, Chin R. Facial granulomas with nasal destruction. Chest1988; 93:422–423.
Sudip Kumar Ghosh, MD, DNB Assistant Professor, Department of Dermatology, Venereology, and Leprosy, R. G. Kar Medical College, Kolkata, India
Debarbrata Bandyopadhyay, MD Professor, Department of Dermatology, Venereology, and Leprosy, R. G. Kar Medical College, Kolkata, India
Loknath Ghoshal, MD Resident Medical Officer-cum-Clinical Tutor, Department of Dermatology, Venereology, and Leprosy, R. G. Kar Medical College, Kolkata, India
Address: Sudip Kumar Ghosh, MD, DNB, Department of Dermatology, Venereology, and Leprosy, R. G. Kar Medical College, 1, Khudiram Bose Sarani, 700004 Kolkata, India; e-mail [email protected]
Sudip Kumar Ghosh, MD, DNB Assistant Professor, Department of Dermatology, Venereology, and Leprosy, R. G. Kar Medical College, Kolkata, India
Debarbrata Bandyopadhyay, MD Professor, Department of Dermatology, Venereology, and Leprosy, R. G. Kar Medical College, Kolkata, India
Loknath Ghoshal, MD Resident Medical Officer-cum-Clinical Tutor, Department of Dermatology, Venereology, and Leprosy, R. G. Kar Medical College, Kolkata, India
Address: Sudip Kumar Ghosh, MD, DNB, Department of Dermatology, Venereology, and Leprosy, R. G. Kar Medical College, 1, Khudiram Bose Sarani, 700004 Kolkata, India; e-mail [email protected]
Author and Disclosure Information
Sudip Kumar Ghosh, MD, DNB Assistant Professor, Department of Dermatology, Venereology, and Leprosy, R. G. Kar Medical College, Kolkata, India
Debarbrata Bandyopadhyay, MD Professor, Department of Dermatology, Venereology, and Leprosy, R. G. Kar Medical College, Kolkata, India
Loknath Ghoshal, MD Resident Medical Officer-cum-Clinical Tutor, Department of Dermatology, Venereology, and Leprosy, R. G. Kar Medical College, Kolkata, India
Address: Sudip Kumar Ghosh, MD, DNB, Department of Dermatology, Venereology, and Leprosy, R. G. Kar Medical College, 1, Khudiram Bose Sarani, 700004 Kolkata, India; e-mail [email protected]
A 12-year-old boy presents with painless swelling and ulceration on and around his nose that has progressed gradually over the last 6 months. The lesion has increased in size despite treatment with topical neomycin and oral erythromycin. He has no systemic symptoms.
Figure 1. Ulcerated plaque with destruction of the right nasal wing.On examination (Figure 1), we note an indurated, nontender plaque with scarring at places on his right cheek, nose, and the vermilion border of the lip. In addition, there are two purulent ulcerations on the nose partly destroying the right nasal wing. The upper lip is also infiltrated, studded with a solitary ulceration. There is no regional lymphadenopathy. An examination of systems is normal.
Cutaneous tuberculosis occurs in many forms, and lupus vulgaris is one of the most common.1 Lupus vulgaris usually arises as a result of hematogenous spread from an endogenous source. It may also arise from exogenous inoculation or as a complication of vaccination with bacille Calmette-Guérin.2
Several morphologic variants have been described.1,2 One form is characterized by plaques, often studded with psoriasiform scales. Large plaques may show irregular areas of scarring with islands of active lupus tissue and a thickened and hyperkeratotic margin. Ulcerative and mutilating variants of lupus vulgaris are characterized by scarring, ulceration, crusts over areas of necrosis, and destruction of the deep tissues and cartilage, resulting in deformities. The vegetative form produces marked infiltration, ulceration, and necrosis, with minimal scarring. Mucous membranes and cartilages are often destroyed. Tumor-like hypertrophic lesions and multiple papular and nodular lesions may also be seen. Nasal lesions may start as nodules, which may bleed and then ulcerate, sometimes resulting in cartilage destruction.
CLINICAL FEATURES AND LABORATORY WORKUP CLINCHED THE DIAGNOSIS
A number of factors helped to confirm the diagnosis in this patient:
A strongly positive Mantoux test (22-mm induration at 48 hours)
Acid-fast bacilli on Ziehl-Neelsen staining of the smear taken from the purulent ulceration
Isolation of Mycobacterium tuberculosis from the purulent exudates via culture in Lowenstein-Jensen medium
Figure 2. Epithelioid cell granuloma and giant cells (hematoxylin and eosin, × 100).A suggestive histopathologic picture (Figure 2)
The features on presentation
A significant clinical improvement within 2 months of starting antituberculosis therapy.
DIFFERENTIAL DIAGNOSIS
The differential diagnosis includes all the conditions in the question above. However, the absence of respiratory and renal involvement helps rule out Wegener granulomatosis; the absence of impaired sensation and nerve thickening helps rule out Hansen disease; and the absence of a nasal septal defect helps rule out Wegener granulomatosis, midline lethal granuloma, and Hansen disease.
On the other hand, the lupoid form of cutaneous leishmaniasis usually presents as an erythematous, infiltrated plaque that often closely resembles lupus vulgaris, but these lesions are usually less destructive than lupus vulgaris. However, the laboratory workup including the microbiological and histopathologic examination clearly excluded the other potential diagnoses in this patient.
TREATMENT
Lupus vulgaris is treated with standard antituberculosis therapy.3 The first phase of a fourdrug regimen is given for 2 months—isoniazid, rifampin (Rifadin), pyrazinamide, and ethambutol (Myambutol). The second phase consists of isoniazid and rifampin for 4 months.3
Early recognition and confirmation of the diagnosis followed by treatment are of immense importance for preventing permanent disfigurement.
A 12-year-old boy presents with painless swelling and ulceration on and around his nose that has progressed gradually over the last 6 months. The lesion has increased in size despite treatment with topical neomycin and oral erythromycin. He has no systemic symptoms.
Figure 1. Ulcerated plaque with destruction of the right nasal wing.On examination (Figure 1), we note an indurated, nontender plaque with scarring at places on his right cheek, nose, and the vermilion border of the lip. In addition, there are two purulent ulcerations on the nose partly destroying the right nasal wing. The upper lip is also infiltrated, studded with a solitary ulceration. There is no regional lymphadenopathy. An examination of systems is normal.
Cutaneous tuberculosis occurs in many forms, and lupus vulgaris is one of the most common.1 Lupus vulgaris usually arises as a result of hematogenous spread from an endogenous source. It may also arise from exogenous inoculation or as a complication of vaccination with bacille Calmette-Guérin.2
Several morphologic variants have been described.1,2 One form is characterized by plaques, often studded with psoriasiform scales. Large plaques may show irregular areas of scarring with islands of active lupus tissue and a thickened and hyperkeratotic margin. Ulcerative and mutilating variants of lupus vulgaris are characterized by scarring, ulceration, crusts over areas of necrosis, and destruction of the deep tissues and cartilage, resulting in deformities. The vegetative form produces marked infiltration, ulceration, and necrosis, with minimal scarring. Mucous membranes and cartilages are often destroyed. Tumor-like hypertrophic lesions and multiple papular and nodular lesions may also be seen. Nasal lesions may start as nodules, which may bleed and then ulcerate, sometimes resulting in cartilage destruction.
CLINICAL FEATURES AND LABORATORY WORKUP CLINCHED THE DIAGNOSIS
A number of factors helped to confirm the diagnosis in this patient:
A strongly positive Mantoux test (22-mm induration at 48 hours)
Acid-fast bacilli on Ziehl-Neelsen staining of the smear taken from the purulent ulceration
Isolation of Mycobacterium tuberculosis from the purulent exudates via culture in Lowenstein-Jensen medium
Figure 2. Epithelioid cell granuloma and giant cells (hematoxylin and eosin, × 100).A suggestive histopathologic picture (Figure 2)
The features on presentation
A significant clinical improvement within 2 months of starting antituberculosis therapy.
DIFFERENTIAL DIAGNOSIS
The differential diagnosis includes all the conditions in the question above. However, the absence of respiratory and renal involvement helps rule out Wegener granulomatosis; the absence of impaired sensation and nerve thickening helps rule out Hansen disease; and the absence of a nasal septal defect helps rule out Wegener granulomatosis, midline lethal granuloma, and Hansen disease.
On the other hand, the lupoid form of cutaneous leishmaniasis usually presents as an erythematous, infiltrated plaque that often closely resembles lupus vulgaris, but these lesions are usually less destructive than lupus vulgaris. However, the laboratory workup including the microbiological and histopathologic examination clearly excluded the other potential diagnoses in this patient.
TREATMENT
Lupus vulgaris is treated with standard antituberculosis therapy.3 The first phase of a fourdrug regimen is given for 2 months—isoniazid, rifampin (Rifadin), pyrazinamide, and ethambutol (Myambutol). The second phase consists of isoniazid and rifampin for 4 months.3
Early recognition and confirmation of the diagnosis followed by treatment are of immense importance for preventing permanent disfigurement.
References
Freitag DS, Chin R. Facial granulomas with nasal destruction. Chest1988; 93:422–423.
A Good-quality patient-oriented evidence B Inconsistent or limited-quality patient-oriented evidence C Consensus, usual practice, opinion, disease-oriented evidence, case series
A 12-Year-old girl came into our hospital for treatment of multiple bumps that had developed around her eyes and other areas of her face 2 months earlier. She had difficulty opening her eyes and complained of gradual weight loss.
On examination, we noted numerous skin-colored, shiny, dome-shaped, coalescing papules and nodules with central umbilications that were distributed mostly on her periocular and perinasal areas (FIGURE). When we expressed the papules with forceps, they exuded a cheesy material. We also noticed crusting and signs of inflammation on her eyelids.
The systemic examination was unremarkable.
FIGURE Opening her eyes was difficult
This 12-year-old patient had multiple dome-shaped, coalescing papules and nodules with central umbilications in the periocular and perinasal areas.
WHAT IS YOUR DIAGNOSIS? HOW WOULD YOU TREAT THIS PATIENT?
Diagnosis: Giant molluscum contagiosum
Molluscum contagiosum (MC) is a relatively common, benign, viral cutaneous infection that primarily affects children, sexually active adults, and immunodeficient individuals. MC accounts for approximately 1% of all diagnosed skin disorders in the United States; internationally, however, the incidence is higher.1 The causative organism of MC is a member of the Poxviridae family2 and is thought to be transmitted by close personal contact, autoinoculation, and fomites.3
FAST TRACK
Molluscum contagiosum is an ocular complication of AIDS.
MC is clinically characterized by the presence of pearly white, dome-shaped papules or nodules with central dells. The lesions are typically located on the trunk, body folds, extremities, and genitalia (particularly when the infection is sexually acquired).2,3 Pruritus and an eczematous reaction can develop around the lesions.
MC is a recognized ocular complication of acquired immune deficiency syndrome (AIDS). Periocular MC can also occur after eyebrow shaping in beauty salons.4 In human immunodeficiency virus (HIV)-positive patients, lesions are usually widespread, tend to be large, and usually occur during the advanced stage of HIV infection.2,5
The differential includes carcinoma When considering a diagnosis of MC, you’ll need to rule out the following causes of similar-looking papules and nodules:
Nodular basal cell carcinoma presents as a slow-growing, firm, shiny, pearly nodule with fine telangiectasia. It may also present as a cystic lesion that can be mistaken for inclusion cysts of the eyelid. If left untreated, the tumor may ulcerate.
Juvenile xanthogranulomas are rubbery, tan-orange papules or nodules. Patients may have one or several papules or nodules in the head and neck region; these lesions may appear elsewhere, as well.
Cryptococcosis may present as painless papules or pustules, which then become nodules that may ulcerate. The lesions may show central umbilications.
Keratoacanthoma begins as a firm, roundish, skin-colored or reddish papule that rapidly progresses to a dome-shaped nodule, with a smooth, shiny surface and a central crateriform ulceration or keratin plug. Patients typically have a solitary lesion that may appear on the face, neck, or dorsum of the upper extremities.
Penicillosis often presents with MC-like skin lesions, in addition to fever, anemia, hepatomegaly, lymphadenopathy, and productive cough.
History and lab work clinch the Dx Diagnosis is made by the distinctive clinical appearance, but can be confirmed by skin biopsy demonstrating eosinophilic molluscum bodies packed into the cells of the spinous layer of the epidermis.3 Giemsa stain of the material obtained from a crushed papule will reveal the presence of pathognomonic “molluscum bodies” in the cells of the epidermis.2,3
Our patient’s Giemsa stain revealed molluscum bodies. And since it is always wise to rule out concomitant HIV infection in patients who have giant MC, we tested our patient. Her results were positive; she had a CD4+ count of 93 cells/mm3.
Many treatment options from which to choose MC is usually self-limiting, although it can take several months—or even a few years—to resolve on its own6 (strength of recommendation [SOR]: B). However, most patients with MC should receive treatment to obtain relief from symptoms, prevent autoinoculation or transmission to close contacts, decrease occurrence of scarring, reduce secondary bacterial infections, and improve cosmesis.
Several treatment options are available, and most rely on destruction of the lesions. Manual extrusion is a simple but effective therapy6 (SOR: B). Cryotherapy and curettage are also effective treatment options5 (SOR: C). Pretreatment topical anesthesia is often helpful if these therapies are used in children.
Topical imiquimod2 (1%-5%) cream applied 3 to 7 times a week can be used to treat generalized MC infection or MC localized to the anogenital area6 (SOR: A). Some patients may improve with topical tretinoin therapy6 (SOR: C).
Chemical cauterization with 10% povidone iodine with 50% salicylic acid7 (SOR: B), 10% potassium hydroxide8 (SOR: B), cantharidin2 (SOR: C), or 25% to 50% trichloroacetic acid6 (SOR: C) is also effective. Treatment with flashlamp pulsed dye laser is a safe and efficient treatment modality9 (SOR: C). Cidofovir10 (1%-3%) cream or ointment, electron beam therapy, and photodynamic therapy have also been used with variable success rates6 (SOR: C).
MC is particularly difficult to treat in patients with poorly managed HIV and AIDS. Pairing proper antiretroviral therapy with lesion-destroying therapies is usually helpful for these patients.3
FAST TRACK
Our patient’s mother turned out to be HIV positive, as well.
If you are caring for a patient with giant MC, you’ll need to stress the benign—but potentially contagious—nature of the disease. Tell the patient to wash his or her hands frequently, to avoid scratching the lesions, and to keep infected areas covered with clothing (when possible). In suspected sexually transmitted cases, the patient should adopt safe sexual practices or abstinence, if necessary. It is unclear whether condoms or other barrier methods provide adequate protection.1
Our patient transfers to the HIV clinic
We sequentially expressed the large lesions on our patient’s face and put her on a course of cefadroxil to control the secondary infection of MC. Her facial lesions gradually improved over 2 months.
We also referred the patient to our institution’s HIV clinic, where she was put on highly active antiretroviral therapy (HAART). We advised her mother to get tested for HIV, and she turned out to be HIV positive, as well.
CORRESPONDENCE Sudip Kumar Ghosh, MD, DNB, Department of Dermatology, Venereology, and Leprosy, R.G. Kar Medical College, 1 Khudiram Bose Sarani, Kolkata-700004, West Bengal, India; [email protected]
References
1. Taillac PP. Molluscum contagiosum: eMedicine, Emergency Medicine. Available at: . Accessed October 29, 2010.
2. Tom W, Friedlander SF. Poxvirus infections. In: Wolff K, Goldsmith LA, Katz SI, et al, eds. Fitzpatrick’s Dermatology in General Medicine. 7th ed. New York, NY: McGraw-Hill; 2008: 1899-1913.
3. Turchin I, Barankin B. Dermacase. Molluscum contagiosum. Can Fam Physician. 2006;52:1395-1407.
4. Ghosh SK, Bandyopadhyay D. Molluscum contagiosum after eyebrow shaping: a beauty salon hazard. Clin Exp Dermatol. 2009;34:e339-e340.
5. Gur I. The epidemiology of molluscum contagiosum in HIVseropositive patients: a unique entity or insignificant finding? Int J STD AIDS. 2008;19:503-506.
7. Ohkuma M. Molluscum contagiosum treated with iodine solution and salicylic acid plaster. Int J Dermatol. 1990;29:443-445.
8. Mahajan BB, Pall A, Gupta RR. Topical 20% KOH—an effective therapeutic modality for molluscum contagiosum in children. Indian J Dermatol Venereol Leprol. 2003;69:175-177.
9. Binder B, Weger W, Komericki P, et al. Treatment of molluscum contagiosum with a pulsed dye laser: pilot study with 19 children. J Dtsch Dermatol Ges. 2008;6:121-125.
10. Watanabe T, Tamaki K. Cidofovir diphosphate inhibits molluscum contagiosum virus DNA polymerase activity. J Invest Dermatol. 2008;128:1327-1329.
Sudip Kumar Ghosh, MD, DNB Debabrata Bandyopadhyay, MD Rajesh Kumar Mandal, MBBS Department of Dermatology, Venereology, and Leprosy, R.G. Kar Medical College, Kolkata, West Bengal, India [email protected]
DEPARTMENT EDITOR Richard P. Usatine, MD University of Texas Health Science Center at San Antonio
The authors reported no potential conflict of interest relevant to this article.
Sudip Kumar Ghosh, MD, DNB Debabrata Bandyopadhyay, MD Rajesh Kumar Mandal, MBBS Department of Dermatology, Venereology, and Leprosy, R.G. Kar Medical College, Kolkata, West Bengal, India [email protected]
DEPARTMENT EDITOR Richard P. Usatine, MD University of Texas Health Science Center at San Antonio
The authors reported no potential conflict of interest relevant to this article.
Author and Disclosure Information
Sudip Kumar Ghosh, MD, DNB Debabrata Bandyopadhyay, MD Rajesh Kumar Mandal, MBBS Department of Dermatology, Venereology, and Leprosy, R.G. Kar Medical College, Kolkata, West Bengal, India [email protected]
DEPARTMENT EDITOR Richard P. Usatine, MD University of Texas Health Science Center at San Antonio
The authors reported no potential conflict of interest relevant to this article.
A Good-quality patient-oriented evidence B Inconsistent or limited-quality patient-oriented evidence C Consensus, usual practice, opinion, disease-oriented evidence, case series
A 12-Year-old girl came into our hospital for treatment of multiple bumps that had developed around her eyes and other areas of her face 2 months earlier. She had difficulty opening her eyes and complained of gradual weight loss.
On examination, we noted numerous skin-colored, shiny, dome-shaped, coalescing papules and nodules with central umbilications that were distributed mostly on her periocular and perinasal areas (FIGURE). When we expressed the papules with forceps, they exuded a cheesy material. We also noticed crusting and signs of inflammation on her eyelids.
The systemic examination was unremarkable.
FIGURE Opening her eyes was difficult
This 12-year-old patient had multiple dome-shaped, coalescing papules and nodules with central umbilications in the periocular and perinasal areas.
WHAT IS YOUR DIAGNOSIS? HOW WOULD YOU TREAT THIS PATIENT?
Diagnosis: Giant molluscum contagiosum
Molluscum contagiosum (MC) is a relatively common, benign, viral cutaneous infection that primarily affects children, sexually active adults, and immunodeficient individuals. MC accounts for approximately 1% of all diagnosed skin disorders in the United States; internationally, however, the incidence is higher.1 The causative organism of MC is a member of the Poxviridae family2 and is thought to be transmitted by close personal contact, autoinoculation, and fomites.3
FAST TRACK
Molluscum contagiosum is an ocular complication of AIDS.
MC is clinically characterized by the presence of pearly white, dome-shaped papules or nodules with central dells. The lesions are typically located on the trunk, body folds, extremities, and genitalia (particularly when the infection is sexually acquired).2,3 Pruritus and an eczematous reaction can develop around the lesions.
MC is a recognized ocular complication of acquired immune deficiency syndrome (AIDS). Periocular MC can also occur after eyebrow shaping in beauty salons.4 In human immunodeficiency virus (HIV)-positive patients, lesions are usually widespread, tend to be large, and usually occur during the advanced stage of HIV infection.2,5
The differential includes carcinoma When considering a diagnosis of MC, you’ll need to rule out the following causes of similar-looking papules and nodules:
Nodular basal cell carcinoma presents as a slow-growing, firm, shiny, pearly nodule with fine telangiectasia. It may also present as a cystic lesion that can be mistaken for inclusion cysts of the eyelid. If left untreated, the tumor may ulcerate.
Juvenile xanthogranulomas are rubbery, tan-orange papules or nodules. Patients may have one or several papules or nodules in the head and neck region; these lesions may appear elsewhere, as well.
Cryptococcosis may present as painless papules or pustules, which then become nodules that may ulcerate. The lesions may show central umbilications.
Keratoacanthoma begins as a firm, roundish, skin-colored or reddish papule that rapidly progresses to a dome-shaped nodule, with a smooth, shiny surface and a central crateriform ulceration or keratin plug. Patients typically have a solitary lesion that may appear on the face, neck, or dorsum of the upper extremities.
Penicillosis often presents with MC-like skin lesions, in addition to fever, anemia, hepatomegaly, lymphadenopathy, and productive cough.
History and lab work clinch the Dx Diagnosis is made by the distinctive clinical appearance, but can be confirmed by skin biopsy demonstrating eosinophilic molluscum bodies packed into the cells of the spinous layer of the epidermis.3 Giemsa stain of the material obtained from a crushed papule will reveal the presence of pathognomonic “molluscum bodies” in the cells of the epidermis.2,3
Our patient’s Giemsa stain revealed molluscum bodies. And since it is always wise to rule out concomitant HIV infection in patients who have giant MC, we tested our patient. Her results were positive; she had a CD4+ count of 93 cells/mm3.
Many treatment options from which to choose MC is usually self-limiting, although it can take several months—or even a few years—to resolve on its own6 (strength of recommendation [SOR]: B). However, most patients with MC should receive treatment to obtain relief from symptoms, prevent autoinoculation or transmission to close contacts, decrease occurrence of scarring, reduce secondary bacterial infections, and improve cosmesis.
Several treatment options are available, and most rely on destruction of the lesions. Manual extrusion is a simple but effective therapy6 (SOR: B). Cryotherapy and curettage are also effective treatment options5 (SOR: C). Pretreatment topical anesthesia is often helpful if these therapies are used in children.
Topical imiquimod2 (1%-5%) cream applied 3 to 7 times a week can be used to treat generalized MC infection or MC localized to the anogenital area6 (SOR: A). Some patients may improve with topical tretinoin therapy6 (SOR: C).
Chemical cauterization with 10% povidone iodine with 50% salicylic acid7 (SOR: B), 10% potassium hydroxide8 (SOR: B), cantharidin2 (SOR: C), or 25% to 50% trichloroacetic acid6 (SOR: C) is also effective. Treatment with flashlamp pulsed dye laser is a safe and efficient treatment modality9 (SOR: C). Cidofovir10 (1%-3%) cream or ointment, electron beam therapy, and photodynamic therapy have also been used with variable success rates6 (SOR: C).
MC is particularly difficult to treat in patients with poorly managed HIV and AIDS. Pairing proper antiretroviral therapy with lesion-destroying therapies is usually helpful for these patients.3
FAST TRACK
Our patient’s mother turned out to be HIV positive, as well.
If you are caring for a patient with giant MC, you’ll need to stress the benign—but potentially contagious—nature of the disease. Tell the patient to wash his or her hands frequently, to avoid scratching the lesions, and to keep infected areas covered with clothing (when possible). In suspected sexually transmitted cases, the patient should adopt safe sexual practices or abstinence, if necessary. It is unclear whether condoms or other barrier methods provide adequate protection.1
Our patient transfers to the HIV clinic
We sequentially expressed the large lesions on our patient’s face and put her on a course of cefadroxil to control the secondary infection of MC. Her facial lesions gradually improved over 2 months.
We also referred the patient to our institution’s HIV clinic, where she was put on highly active antiretroviral therapy (HAART). We advised her mother to get tested for HIV, and she turned out to be HIV positive, as well.
CORRESPONDENCE Sudip Kumar Ghosh, MD, DNB, Department of Dermatology, Venereology, and Leprosy, R.G. Kar Medical College, 1 Khudiram Bose Sarani, Kolkata-700004, West Bengal, India; [email protected]
Strength of recommendation (SOR)
A Good-quality patient-oriented evidence B Inconsistent or limited-quality patient-oriented evidence C Consensus, usual practice, opinion, disease-oriented evidence, case series
A 12-Year-old girl came into our hospital for treatment of multiple bumps that had developed around her eyes and other areas of her face 2 months earlier. She had difficulty opening her eyes and complained of gradual weight loss.
On examination, we noted numerous skin-colored, shiny, dome-shaped, coalescing papules and nodules with central umbilications that were distributed mostly on her periocular and perinasal areas (FIGURE). When we expressed the papules with forceps, they exuded a cheesy material. We also noticed crusting and signs of inflammation on her eyelids.
The systemic examination was unremarkable.
FIGURE Opening her eyes was difficult
This 12-year-old patient had multiple dome-shaped, coalescing papules and nodules with central umbilications in the periocular and perinasal areas.
WHAT IS YOUR DIAGNOSIS? HOW WOULD YOU TREAT THIS PATIENT?
Diagnosis: Giant molluscum contagiosum
Molluscum contagiosum (MC) is a relatively common, benign, viral cutaneous infection that primarily affects children, sexually active adults, and immunodeficient individuals. MC accounts for approximately 1% of all diagnosed skin disorders in the United States; internationally, however, the incidence is higher.1 The causative organism of MC is a member of the Poxviridae family2 and is thought to be transmitted by close personal contact, autoinoculation, and fomites.3
FAST TRACK
Molluscum contagiosum is an ocular complication of AIDS.
MC is clinically characterized by the presence of pearly white, dome-shaped papules or nodules with central dells. The lesions are typically located on the trunk, body folds, extremities, and genitalia (particularly when the infection is sexually acquired).2,3 Pruritus and an eczematous reaction can develop around the lesions.
MC is a recognized ocular complication of acquired immune deficiency syndrome (AIDS). Periocular MC can also occur after eyebrow shaping in beauty salons.4 In human immunodeficiency virus (HIV)-positive patients, lesions are usually widespread, tend to be large, and usually occur during the advanced stage of HIV infection.2,5
The differential includes carcinoma When considering a diagnosis of MC, you’ll need to rule out the following causes of similar-looking papules and nodules:
Nodular basal cell carcinoma presents as a slow-growing, firm, shiny, pearly nodule with fine telangiectasia. It may also present as a cystic lesion that can be mistaken for inclusion cysts of the eyelid. If left untreated, the tumor may ulcerate.
Juvenile xanthogranulomas are rubbery, tan-orange papules or nodules. Patients may have one or several papules or nodules in the head and neck region; these lesions may appear elsewhere, as well.
Cryptococcosis may present as painless papules or pustules, which then become nodules that may ulcerate. The lesions may show central umbilications.
Keratoacanthoma begins as a firm, roundish, skin-colored or reddish papule that rapidly progresses to a dome-shaped nodule, with a smooth, shiny surface and a central crateriform ulceration or keratin plug. Patients typically have a solitary lesion that may appear on the face, neck, or dorsum of the upper extremities.
Penicillosis often presents with MC-like skin lesions, in addition to fever, anemia, hepatomegaly, lymphadenopathy, and productive cough.
History and lab work clinch the Dx Diagnosis is made by the distinctive clinical appearance, but can be confirmed by skin biopsy demonstrating eosinophilic molluscum bodies packed into the cells of the spinous layer of the epidermis.3 Giemsa stain of the material obtained from a crushed papule will reveal the presence of pathognomonic “molluscum bodies” in the cells of the epidermis.2,3
Our patient’s Giemsa stain revealed molluscum bodies. And since it is always wise to rule out concomitant HIV infection in patients who have giant MC, we tested our patient. Her results were positive; she had a CD4+ count of 93 cells/mm3.
Many treatment options from which to choose MC is usually self-limiting, although it can take several months—or even a few years—to resolve on its own6 (strength of recommendation [SOR]: B). However, most patients with MC should receive treatment to obtain relief from symptoms, prevent autoinoculation or transmission to close contacts, decrease occurrence of scarring, reduce secondary bacterial infections, and improve cosmesis.
Several treatment options are available, and most rely on destruction of the lesions. Manual extrusion is a simple but effective therapy6 (SOR: B). Cryotherapy and curettage are also effective treatment options5 (SOR: C). Pretreatment topical anesthesia is often helpful if these therapies are used in children.
Topical imiquimod2 (1%-5%) cream applied 3 to 7 times a week can be used to treat generalized MC infection or MC localized to the anogenital area6 (SOR: A). Some patients may improve with topical tretinoin therapy6 (SOR: C).
Chemical cauterization with 10% povidone iodine with 50% salicylic acid7 (SOR: B), 10% potassium hydroxide8 (SOR: B), cantharidin2 (SOR: C), or 25% to 50% trichloroacetic acid6 (SOR: C) is also effective. Treatment with flashlamp pulsed dye laser is a safe and efficient treatment modality9 (SOR: C). Cidofovir10 (1%-3%) cream or ointment, electron beam therapy, and photodynamic therapy have also been used with variable success rates6 (SOR: C).
MC is particularly difficult to treat in patients with poorly managed HIV and AIDS. Pairing proper antiretroviral therapy with lesion-destroying therapies is usually helpful for these patients.3
FAST TRACK
Our patient’s mother turned out to be HIV positive, as well.
If you are caring for a patient with giant MC, you’ll need to stress the benign—but potentially contagious—nature of the disease. Tell the patient to wash his or her hands frequently, to avoid scratching the lesions, and to keep infected areas covered with clothing (when possible). In suspected sexually transmitted cases, the patient should adopt safe sexual practices or abstinence, if necessary. It is unclear whether condoms or other barrier methods provide adequate protection.1
Our patient transfers to the HIV clinic
We sequentially expressed the large lesions on our patient’s face and put her on a course of cefadroxil to control the secondary infection of MC. Her facial lesions gradually improved over 2 months.
We also referred the patient to our institution’s HIV clinic, where she was put on highly active antiretroviral therapy (HAART). We advised her mother to get tested for HIV, and she turned out to be HIV positive, as well.
CORRESPONDENCE Sudip Kumar Ghosh, MD, DNB, Department of Dermatology, Venereology, and Leprosy, R.G. Kar Medical College, 1 Khudiram Bose Sarani, Kolkata-700004, West Bengal, India; [email protected]
References
1. Taillac PP. Molluscum contagiosum: eMedicine, Emergency Medicine. Available at: . Accessed October 29, 2010.
2. Tom W, Friedlander SF. Poxvirus infections. In: Wolff K, Goldsmith LA, Katz SI, et al, eds. Fitzpatrick’s Dermatology in General Medicine. 7th ed. New York, NY: McGraw-Hill; 2008: 1899-1913.
3. Turchin I, Barankin B. Dermacase. Molluscum contagiosum. Can Fam Physician. 2006;52:1395-1407.
4. Ghosh SK, Bandyopadhyay D. Molluscum contagiosum after eyebrow shaping: a beauty salon hazard. Clin Exp Dermatol. 2009;34:e339-e340.
5. Gur I. The epidemiology of molluscum contagiosum in HIVseropositive patients: a unique entity or insignificant finding? Int J STD AIDS. 2008;19:503-506.
7. Ohkuma M. Molluscum contagiosum treated with iodine solution and salicylic acid plaster. Int J Dermatol. 1990;29:443-445.
8. Mahajan BB, Pall A, Gupta RR. Topical 20% KOH—an effective therapeutic modality for molluscum contagiosum in children. Indian J Dermatol Venereol Leprol. 2003;69:175-177.
9. Binder B, Weger W, Komericki P, et al. Treatment of molluscum contagiosum with a pulsed dye laser: pilot study with 19 children. J Dtsch Dermatol Ges. 2008;6:121-125.
10. Watanabe T, Tamaki K. Cidofovir diphosphate inhibits molluscum contagiosum virus DNA polymerase activity. J Invest Dermatol. 2008;128:1327-1329.
References
1. Taillac PP. Molluscum contagiosum: eMedicine, Emergency Medicine. Available at: . Accessed October 29, 2010.
2. Tom W, Friedlander SF. Poxvirus infections. In: Wolff K, Goldsmith LA, Katz SI, et al, eds. Fitzpatrick’s Dermatology in General Medicine. 7th ed. New York, NY: McGraw-Hill; 2008: 1899-1913.
3. Turchin I, Barankin B. Dermacase. Molluscum contagiosum. Can Fam Physician. 2006;52:1395-1407.
4. Ghosh SK, Bandyopadhyay D. Molluscum contagiosum after eyebrow shaping: a beauty salon hazard. Clin Exp Dermatol. 2009;34:e339-e340.
5. Gur I. The epidemiology of molluscum contagiosum in HIVseropositive patients: a unique entity or insignificant finding? Int J STD AIDS. 2008;19:503-506.
7. Ohkuma M. Molluscum contagiosum treated with iodine solution and salicylic acid plaster. Int J Dermatol. 1990;29:443-445.
8. Mahajan BB, Pall A, Gupta RR. Topical 20% KOH—an effective therapeutic modality for molluscum contagiosum in children. Indian J Dermatol Venereol Leprol. 2003;69:175-177.
9. Binder B, Weger W, Komericki P, et al. Treatment of molluscum contagiosum with a pulsed dye laser: pilot study with 19 children. J Dtsch Dermatol Ges. 2008;6:121-125.
10. Watanabe T, Tamaki K. Cidofovir diphosphate inhibits molluscum contagiosum virus DNA polymerase activity. J Invest Dermatol. 2008;128:1327-1329.
