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Purpuric lesions in an elderly woman
A 68-year-old woman presented with a 5-day history of extensive pruritic purpuric skin lesions of varying sizes on her trunk and extremities (FIGURE 1A and 1B). In addition, the patient had a few nonblanching, erythematous macules on her extremities.
The patient had no neurological complaints and her family history was negative for a similar condition. We performed a punch biopsy.
WHAT IS YOUR DIAGNOSIS?
HOW WOULD YOU TREAT THIS PATIENT?
Diagnosis: Churg-Strauss syndrome
Churg-Strauss syndrome (CSS)—also known as allergic granulomatosis and angiitis—is a rare multisystemic vasculitis of small- to medium-sized vessels characterized by asthma, chronic rhinosinusitis, and prominent peripheral blood eosinophilia.1,2 Mean diagnosis age is 50 years with no gender predilection.2 Any organ system can be affected, although the lungs are most commonly involved, followed by the skin.1,2
Based on criteria from the American College of Rheumatology, the diagnosis of CSS can be made if 4 of the following 6 criteria are met: (1) asthma, (2) eosinophilia >10% on a differential white blood cell (WBC) count, (3) paranasal sinus abnormalities, (4) a transient pulmonary infiltrate detected on chest x-ray, (5) mono- or polyneuropathy, and (6) a biopsy specimen showing extravascular accumulation of eosinophils.2
Skin biopsy specimen from our patient showed leukocytoclastic vasculitis with prominent tissue eosinophilia. Laboratory studies showed an elevated WBC count of 12,300/mcL (reference range, 4500-11,000/mcL), and eosinophilia of 40% (reference range, 1%-4%). A serologic test for perinuclear pattern antineutrophil cytoplasmic antibodies (p-ANCA) was positive. (More on this in a moment.) Radiography of the chest showed transient pulmonary infiltrates.
Based on the clinical and laboratory findings, the patient was positive for 4 of 6 criteria and given a diagnosis of CSS.
What we know—and don’t know—about CSS
The exact etiopathogenesis of CSS is unknown.2-4 Although ANCAs are detected in about 40% to 60% of CSS patients, it is not yet known whether ANCAs have a pathogenic role.2-3 Abnormalities in immunologic function also occur, including heightened Th1 and Th2 lymphocyte function, increased recruitment of eosinophils, and decreased eosinophil apoptosis. Genetic factors, including certain interleukin-10 polymorphisms and HLA classes such as HLA-DRB4, may also contribute to CSS pathogenesis.4
Three distinct sequential phases have been described, although these are not always clearly distinguishable.2,5
• The first is the prodromal or allergic phase, which is characterized by the onset of asthma later in life in patients with no family history of atopy. There may or may not be an associated allergic rhinitis.
• In the eosinophilic phase, peripheral blood eosinophilia and eosinophilic infiltration of multiple organs (especially the lungs and gastrointestinal [GI] tract) occur.
• The vasculitis phase is characterized by life-threatening systemic vasculitis of the small and medium vessels that is often associated with vascular and extravascular granulomatosis.
Cutaneous and extracutaneous findings
One-half to two-thirds of patients with CSS have cutaneous manifestations that typically present in the vasculitis phase.2,5 The most common skin finding is palpable purpura on the lower extremities. Macular or papular erythematous eruption, urticaria, subcutaneous skin-colored or erythematous nodules, livedo reticularis, and erythema multiforme–like eruption may also be seen.2,5,6 Skin biopsies will show numerous eosinophils with either leukocytoclastic vasculitis or extravascular necrotizing granuloma.5
Extracutaneous manifestations of CSS include renal, cardiac, GI tract, and nervous system involvement.2,7
To identify patients with a poor prognosis, the 5-factor score (FFS) can be used. This score assigns 1 point each to GI tract involvement, renal insufficiency, proteinuria, central nervous system involvement, and cardiomyopathy.7 CSS patients with an FFS ≥2 have a considerably greater risk of mortality.7
Treatment involves corticosteroids
Systemic corticosteroids (prednisone, 1 mg/kg/day) are the primary treatment for patients with CSS; most patients improve dramatically with therapy.2 Adjunctive therapy with immunosuppressive agents such as cyclophosphamide, methotrexate (10-15 mg per week), chlorambucil, or azathioprine may be needed if a patient does not respond adequately to steroids alone.2
Prednisone for our patient
We started our patient on prednisone 1 mg/kg/d. Her skin lesions resolved and subsequent laboratory tests, including eosinophil counts, normalized. Prednisone therapy was gradually tapered over several months to attain the lowest dose required for control of symptoms—in this case, 5 mg/d.
CORRESPONDENCE
Ossama Abbas, MD, Associate Professor, Department of Dermatology, American University of Beirut Medical Center, PO Box 11-0236, Riad El Solh, Beirut 1107 2020, Beirut, Lebanon; [email protected]
1. Churg J, Strauss L. Allergic granulomatosis, allergic angiitis and periarteritis nodosa. Am J Pathol. 1951;27:277-301.
2. Sinico RA, Bottero P. Churg-Strauss angiitis. Best Pract Res Clin Rheumatol. 2009;23:355-366.
3. Zwerina J, Axmann R, Jatzwauk M, et al. Pathogenesis of Churg-Strauss syndrome: recent insights. Autoimmunity. 2009;42:376-379.
4. Vaglio A, Martorana D, Maggiore U, et al; Secondary and Primary Vasculitis Study Group. HLA-DRB4 as a genetic risk factor for Churg-Strauss syndrome. Arthritis Rheum. 2007;56:3159-3166.
5. Davis MD, Daoud MS, McEvoy MT, et al. Cutaneous manifestations of Churg-Strauss syndrome: a clinicopathologic correlation. J Am Acad Dermatol. 1997;37(2 pt 1):199-203.
6. Tlacuilo-Parra A, Soto-Ortíz JA, Guevara-Gutiérrez E. Churg-Strauss syndrome manifested by urticarial plaques. Int J Dermatol. 2003;42:386-388.
7. Guillevin L, Lhote F, Gayraud M, et al. Prognostic factors in polyarteritis nodosa and Churg-Strauss syndrome. A prospective study in 342 patients. Medicine (Baltimore). 1996;75:17-28.
A 68-year-old woman presented with a 5-day history of extensive pruritic purpuric skin lesions of varying sizes on her trunk and extremities (FIGURE 1A and 1B). In addition, the patient had a few nonblanching, erythematous macules on her extremities.
The patient had no neurological complaints and her family history was negative for a similar condition. We performed a punch biopsy.
WHAT IS YOUR DIAGNOSIS?
HOW WOULD YOU TREAT THIS PATIENT?
Diagnosis: Churg-Strauss syndrome
Churg-Strauss syndrome (CSS)—also known as allergic granulomatosis and angiitis—is a rare multisystemic vasculitis of small- to medium-sized vessels characterized by asthma, chronic rhinosinusitis, and prominent peripheral blood eosinophilia.1,2 Mean diagnosis age is 50 years with no gender predilection.2 Any organ system can be affected, although the lungs are most commonly involved, followed by the skin.1,2
Based on criteria from the American College of Rheumatology, the diagnosis of CSS can be made if 4 of the following 6 criteria are met: (1) asthma, (2) eosinophilia >10% on a differential white blood cell (WBC) count, (3) paranasal sinus abnormalities, (4) a transient pulmonary infiltrate detected on chest x-ray, (5) mono- or polyneuropathy, and (6) a biopsy specimen showing extravascular accumulation of eosinophils.2
Skin biopsy specimen from our patient showed leukocytoclastic vasculitis with prominent tissue eosinophilia. Laboratory studies showed an elevated WBC count of 12,300/mcL (reference range, 4500-11,000/mcL), and eosinophilia of 40% (reference range, 1%-4%). A serologic test for perinuclear pattern antineutrophil cytoplasmic antibodies (p-ANCA) was positive. (More on this in a moment.) Radiography of the chest showed transient pulmonary infiltrates.
Based on the clinical and laboratory findings, the patient was positive for 4 of 6 criteria and given a diagnosis of CSS.
What we know—and don’t know—about CSS
The exact etiopathogenesis of CSS is unknown.2-4 Although ANCAs are detected in about 40% to 60% of CSS patients, it is not yet known whether ANCAs have a pathogenic role.2-3 Abnormalities in immunologic function also occur, including heightened Th1 and Th2 lymphocyte function, increased recruitment of eosinophils, and decreased eosinophil apoptosis. Genetic factors, including certain interleukin-10 polymorphisms and HLA classes such as HLA-DRB4, may also contribute to CSS pathogenesis.4
Three distinct sequential phases have been described, although these are not always clearly distinguishable.2,5
• The first is the prodromal or allergic phase, which is characterized by the onset of asthma later in life in patients with no family history of atopy. There may or may not be an associated allergic rhinitis.
• In the eosinophilic phase, peripheral blood eosinophilia and eosinophilic infiltration of multiple organs (especially the lungs and gastrointestinal [GI] tract) occur.
• The vasculitis phase is characterized by life-threatening systemic vasculitis of the small and medium vessels that is often associated with vascular and extravascular granulomatosis.
Cutaneous and extracutaneous findings
One-half to two-thirds of patients with CSS have cutaneous manifestations that typically present in the vasculitis phase.2,5 The most common skin finding is palpable purpura on the lower extremities. Macular or papular erythematous eruption, urticaria, subcutaneous skin-colored or erythematous nodules, livedo reticularis, and erythema multiforme–like eruption may also be seen.2,5,6 Skin biopsies will show numerous eosinophils with either leukocytoclastic vasculitis or extravascular necrotizing granuloma.5
Extracutaneous manifestations of CSS include renal, cardiac, GI tract, and nervous system involvement.2,7
To identify patients with a poor prognosis, the 5-factor score (FFS) can be used. This score assigns 1 point each to GI tract involvement, renal insufficiency, proteinuria, central nervous system involvement, and cardiomyopathy.7 CSS patients with an FFS ≥2 have a considerably greater risk of mortality.7
Treatment involves corticosteroids
Systemic corticosteroids (prednisone, 1 mg/kg/day) are the primary treatment for patients with CSS; most patients improve dramatically with therapy.2 Adjunctive therapy with immunosuppressive agents such as cyclophosphamide, methotrexate (10-15 mg per week), chlorambucil, or azathioprine may be needed if a patient does not respond adequately to steroids alone.2
Prednisone for our patient
We started our patient on prednisone 1 mg/kg/d. Her skin lesions resolved and subsequent laboratory tests, including eosinophil counts, normalized. Prednisone therapy was gradually tapered over several months to attain the lowest dose required for control of symptoms—in this case, 5 mg/d.
CORRESPONDENCE
Ossama Abbas, MD, Associate Professor, Department of Dermatology, American University of Beirut Medical Center, PO Box 11-0236, Riad El Solh, Beirut 1107 2020, Beirut, Lebanon; [email protected]
A 68-year-old woman presented with a 5-day history of extensive pruritic purpuric skin lesions of varying sizes on her trunk and extremities (FIGURE 1A and 1B). In addition, the patient had a few nonblanching, erythematous macules on her extremities.
The patient had no neurological complaints and her family history was negative for a similar condition. We performed a punch biopsy.
WHAT IS YOUR DIAGNOSIS?
HOW WOULD YOU TREAT THIS PATIENT?
Diagnosis: Churg-Strauss syndrome
Churg-Strauss syndrome (CSS)—also known as allergic granulomatosis and angiitis—is a rare multisystemic vasculitis of small- to medium-sized vessels characterized by asthma, chronic rhinosinusitis, and prominent peripheral blood eosinophilia.1,2 Mean diagnosis age is 50 years with no gender predilection.2 Any organ system can be affected, although the lungs are most commonly involved, followed by the skin.1,2
Based on criteria from the American College of Rheumatology, the diagnosis of CSS can be made if 4 of the following 6 criteria are met: (1) asthma, (2) eosinophilia >10% on a differential white blood cell (WBC) count, (3) paranasal sinus abnormalities, (4) a transient pulmonary infiltrate detected on chest x-ray, (5) mono- or polyneuropathy, and (6) a biopsy specimen showing extravascular accumulation of eosinophils.2
Skin biopsy specimen from our patient showed leukocytoclastic vasculitis with prominent tissue eosinophilia. Laboratory studies showed an elevated WBC count of 12,300/mcL (reference range, 4500-11,000/mcL), and eosinophilia of 40% (reference range, 1%-4%). A serologic test for perinuclear pattern antineutrophil cytoplasmic antibodies (p-ANCA) was positive. (More on this in a moment.) Radiography of the chest showed transient pulmonary infiltrates.
Based on the clinical and laboratory findings, the patient was positive for 4 of 6 criteria and given a diagnosis of CSS.
What we know—and don’t know—about CSS
The exact etiopathogenesis of CSS is unknown.2-4 Although ANCAs are detected in about 40% to 60% of CSS patients, it is not yet known whether ANCAs have a pathogenic role.2-3 Abnormalities in immunologic function also occur, including heightened Th1 and Th2 lymphocyte function, increased recruitment of eosinophils, and decreased eosinophil apoptosis. Genetic factors, including certain interleukin-10 polymorphisms and HLA classes such as HLA-DRB4, may also contribute to CSS pathogenesis.4
Three distinct sequential phases have been described, although these are not always clearly distinguishable.2,5
• The first is the prodromal or allergic phase, which is characterized by the onset of asthma later in life in patients with no family history of atopy. There may or may not be an associated allergic rhinitis.
• In the eosinophilic phase, peripheral blood eosinophilia and eosinophilic infiltration of multiple organs (especially the lungs and gastrointestinal [GI] tract) occur.
• The vasculitis phase is characterized by life-threatening systemic vasculitis of the small and medium vessels that is often associated with vascular and extravascular granulomatosis.
Cutaneous and extracutaneous findings
One-half to two-thirds of patients with CSS have cutaneous manifestations that typically present in the vasculitis phase.2,5 The most common skin finding is palpable purpura on the lower extremities. Macular or papular erythematous eruption, urticaria, subcutaneous skin-colored or erythematous nodules, livedo reticularis, and erythema multiforme–like eruption may also be seen.2,5,6 Skin biopsies will show numerous eosinophils with either leukocytoclastic vasculitis or extravascular necrotizing granuloma.5
Extracutaneous manifestations of CSS include renal, cardiac, GI tract, and nervous system involvement.2,7
To identify patients with a poor prognosis, the 5-factor score (FFS) can be used. This score assigns 1 point each to GI tract involvement, renal insufficiency, proteinuria, central nervous system involvement, and cardiomyopathy.7 CSS patients with an FFS ≥2 have a considerably greater risk of mortality.7
Treatment involves corticosteroids
Systemic corticosteroids (prednisone, 1 mg/kg/day) are the primary treatment for patients with CSS; most patients improve dramatically with therapy.2 Adjunctive therapy with immunosuppressive agents such as cyclophosphamide, methotrexate (10-15 mg per week), chlorambucil, or azathioprine may be needed if a patient does not respond adequately to steroids alone.2
Prednisone for our patient
We started our patient on prednisone 1 mg/kg/d. Her skin lesions resolved and subsequent laboratory tests, including eosinophil counts, normalized. Prednisone therapy was gradually tapered over several months to attain the lowest dose required for control of symptoms—in this case, 5 mg/d.
CORRESPONDENCE
Ossama Abbas, MD, Associate Professor, Department of Dermatology, American University of Beirut Medical Center, PO Box 11-0236, Riad El Solh, Beirut 1107 2020, Beirut, Lebanon; [email protected]
1. Churg J, Strauss L. Allergic granulomatosis, allergic angiitis and periarteritis nodosa. Am J Pathol. 1951;27:277-301.
2. Sinico RA, Bottero P. Churg-Strauss angiitis. Best Pract Res Clin Rheumatol. 2009;23:355-366.
3. Zwerina J, Axmann R, Jatzwauk M, et al. Pathogenesis of Churg-Strauss syndrome: recent insights. Autoimmunity. 2009;42:376-379.
4. Vaglio A, Martorana D, Maggiore U, et al; Secondary and Primary Vasculitis Study Group. HLA-DRB4 as a genetic risk factor for Churg-Strauss syndrome. Arthritis Rheum. 2007;56:3159-3166.
5. Davis MD, Daoud MS, McEvoy MT, et al. Cutaneous manifestations of Churg-Strauss syndrome: a clinicopathologic correlation. J Am Acad Dermatol. 1997;37(2 pt 1):199-203.
6. Tlacuilo-Parra A, Soto-Ortíz JA, Guevara-Gutiérrez E. Churg-Strauss syndrome manifested by urticarial plaques. Int J Dermatol. 2003;42:386-388.
7. Guillevin L, Lhote F, Gayraud M, et al. Prognostic factors in polyarteritis nodosa and Churg-Strauss syndrome. A prospective study in 342 patients. Medicine (Baltimore). 1996;75:17-28.
1. Churg J, Strauss L. Allergic granulomatosis, allergic angiitis and periarteritis nodosa. Am J Pathol. 1951;27:277-301.
2. Sinico RA, Bottero P. Churg-Strauss angiitis. Best Pract Res Clin Rheumatol. 2009;23:355-366.
3. Zwerina J, Axmann R, Jatzwauk M, et al. Pathogenesis of Churg-Strauss syndrome: recent insights. Autoimmunity. 2009;42:376-379.
4. Vaglio A, Martorana D, Maggiore U, et al; Secondary and Primary Vasculitis Study Group. HLA-DRB4 as a genetic risk factor for Churg-Strauss syndrome. Arthritis Rheum. 2007;56:3159-3166.
5. Davis MD, Daoud MS, McEvoy MT, et al. Cutaneous manifestations of Churg-Strauss syndrome: a clinicopathologic correlation. J Am Acad Dermatol. 1997;37(2 pt 1):199-203.
6. Tlacuilo-Parra A, Soto-Ortíz JA, Guevara-Gutiérrez E. Churg-Strauss syndrome manifested by urticarial plaques. Int J Dermatol. 2003;42:386-388.
7. Guillevin L, Lhote F, Gayraud M, et al. Prognostic factors in polyarteritis nodosa and Churg-Strauss syndrome. A prospective study in 342 patients. Medicine (Baltimore). 1996;75:17-28.
Recurrent vesicular eruption on the right hand
The parents of an 8-year-old boy brought their son to our clinic because they were worried about the recurrent painful lesions on the pinkie, ring, and middle fingers of his right hand (FIGURE 1A and 1B). The lesions, which had recurred monthly for the past 3 years, typically lasted a few days and then spontaneously resolved.
WHAT IS YOUR DIAGNOSIS?
HOW WOULD YOU TREAT THIS PATIENT?
The diagnosis: Herpetic whitlow
Herpetic whitlow, or herpes simplex virus (HSV) infection of the hand, was first reported by Adamson in 1909.1 Herpes infection of the hand classically has a bimodal age distribution. It may be seen in children younger than 10 years of age or in adults between 20 and 30 years of age.2 In children, it is caused almost exclusively by HSV-1, whereas in adults it can be caused by HSV-1 or HSV-2.2,3
HSV infection of the hand classically occurs as a result of autoinoculation following herpetic gingivostomatitis. After inoculation, the virus has an incubation period of 2 to 20 days before vesicles appear.4 The appearance of the lesions is associated with intense throbbing pain. Fever and systemic symptoms are rare.
What you’ll see. Patients with herpetic whitlow will develop a single vesicle or cluster of vesicles on a single digit a few days after their skin has been irritated or exposed to minor trauma.2,4 Vesicles are typically clear in color and have an erythematous base. However, they are often superinfected with bacteria and may exhibit signs of impetiginization. The most common location of the vesicles is on the terminal phalanx of the thumb, index, or middle finger.3
Differential includes dactylitis
Painful fingers may also be suggestive of dactylitis.
Blistering distal dactylitis, mostly caused by group A β-hemolytic streptococci, is a bacterial infection that manifests as a tense bullae over the anterior fat pad of the volar aspect of the distal part of a single finger (or rarely a toe); diagnosis is usually confirmed by culture.5
Sickle cell dactylitis, or hand-foot syndrome, is caused by localized bone marrow infarction of the carpal and tarsal bones and phalanges. Patients will complain of a sudden onset of warm, tender global swelling of the hands and/or feet that is occasionally accompanied by fever and leucocytosis.5
Spondyloarthritis dactylitis, or a “sausage-like” digit, is usually caused by flexor tenosynovitis and presents as diffuse painful swelling of the fingers and toes, mainly over the flexor tendons.5
Making the diagnosis
The diagnosis of herpetic whitlow is clinical. If the diagnosis is unclear, diagnostic tests can include viral culture, serum antibody titers, a Tzanck smear, lesion specimen antigen testing, or histopathologic examinations. In this case, swab cultures revealed moderate growth of group A b-hemolytic streptococci. Fungal smears and cultures were negative. Histopathology revealed intraepidermal vesiculation with ballooning and reticular degeneration and cytopathic changes of herpetic infection.
The natural history of the untreated, uncomplicated herpetic whitlow is complete clearance within 2 to 3 weeks.4 Rare complications include systemic viremia, ocular infection, nail dystrophy, nail loss, scarring, and localized hyperesthesia or hypoesthesia.2,4,6,7
Treatment with acyclovir
Oral acyclovir (2 g/day in 3 doses for 10 days) taken during the prodromal stage of recurrent HSV-2 herpetic whitlow has been shown to reduce the duration of symptoms from 10.1 to 3.7 days.8 Prophylactic use of oral acyclovir (200 mg, 4 times daily for up to 2 years) has been shown to be effective in suppressing recurrent HSV infection of nongenital skin.9
A good outcome
Our patient was given a 10-day course of acyclovir 400 mg orally 3 times daily and cefadroxil 500 mg orally twice daily (for superimposed bacterial infection). The lesions had completely disappeared upon follow-up 2 weeks later.
CORRESPONDENCE
Ossama Abbas, MD, American University of Beirut Medical Center, Riad El Solh/Beirut, Lebanon; [email protected]
1. Adamson HG. Herpes febrilis attacking the fingers. Br J Dermatol. 1909;21:323-324.
2. Feder HM Jr, Long SS. Herpetic whitlow. Epidemiology, clinical characteristics, diagnosis, and treatment. Am J Dis Child. 1983;137:861-863.
3. Gill MJ, Arlette J, Tyrrell DL, et al. Herpes simplex virus infection of the hand. Clinical features and management. Am J Med. 1988;85:53-56.
4. Haedicke GJ, Grossman JA, Fisher AE. Herpetic whitlow of the digits. J Hand Surg Br. 1989;14:443-446.
5. Olivieri I, Scarano E, Padula A, et al. Dactylitis, a term for different digit diseases. Scand J Rheumatol. 2006;35:333-340.
6. Stern H, Elek SD, Millar DM, et al. Herpetic whitlow, a form of cross-infection in hospitals. Lancet. 1959;2:871-874.
7. Fowler JR. Viral infections. Hand Clin. 1989;5:613-627.
8. Gill MJ, Bryant HE. Oral acyclovir therapy of recurrent herpes simplex virus type 2 infection of the hand. Anitmicrob Agents Chemother. 1991;35:382-383.
9. Rubright JH, Shafritz AB. The herpetic whitlow. J Hand Surg Am. 2011;36:340-342.
The parents of an 8-year-old boy brought their son to our clinic because they were worried about the recurrent painful lesions on the pinkie, ring, and middle fingers of his right hand (FIGURE 1A and 1B). The lesions, which had recurred monthly for the past 3 years, typically lasted a few days and then spontaneously resolved.
WHAT IS YOUR DIAGNOSIS?
HOW WOULD YOU TREAT THIS PATIENT?
The diagnosis: Herpetic whitlow
Herpetic whitlow, or herpes simplex virus (HSV) infection of the hand, was first reported by Adamson in 1909.1 Herpes infection of the hand classically has a bimodal age distribution. It may be seen in children younger than 10 years of age or in adults between 20 and 30 years of age.2 In children, it is caused almost exclusively by HSV-1, whereas in adults it can be caused by HSV-1 or HSV-2.2,3
HSV infection of the hand classically occurs as a result of autoinoculation following herpetic gingivostomatitis. After inoculation, the virus has an incubation period of 2 to 20 days before vesicles appear.4 The appearance of the lesions is associated with intense throbbing pain. Fever and systemic symptoms are rare.
What you’ll see. Patients with herpetic whitlow will develop a single vesicle or cluster of vesicles on a single digit a few days after their skin has been irritated or exposed to minor trauma.2,4 Vesicles are typically clear in color and have an erythematous base. However, they are often superinfected with bacteria and may exhibit signs of impetiginization. The most common location of the vesicles is on the terminal phalanx of the thumb, index, or middle finger.3
Differential includes dactylitis
Painful fingers may also be suggestive of dactylitis.
Blistering distal dactylitis, mostly caused by group A β-hemolytic streptococci, is a bacterial infection that manifests as a tense bullae over the anterior fat pad of the volar aspect of the distal part of a single finger (or rarely a toe); diagnosis is usually confirmed by culture.5
Sickle cell dactylitis, or hand-foot syndrome, is caused by localized bone marrow infarction of the carpal and tarsal bones and phalanges. Patients will complain of a sudden onset of warm, tender global swelling of the hands and/or feet that is occasionally accompanied by fever and leucocytosis.5
Spondyloarthritis dactylitis, or a “sausage-like” digit, is usually caused by flexor tenosynovitis and presents as diffuse painful swelling of the fingers and toes, mainly over the flexor tendons.5
Making the diagnosis
The diagnosis of herpetic whitlow is clinical. If the diagnosis is unclear, diagnostic tests can include viral culture, serum antibody titers, a Tzanck smear, lesion specimen antigen testing, or histopathologic examinations. In this case, swab cultures revealed moderate growth of group A b-hemolytic streptococci. Fungal smears and cultures were negative. Histopathology revealed intraepidermal vesiculation with ballooning and reticular degeneration and cytopathic changes of herpetic infection.
The natural history of the untreated, uncomplicated herpetic whitlow is complete clearance within 2 to 3 weeks.4 Rare complications include systemic viremia, ocular infection, nail dystrophy, nail loss, scarring, and localized hyperesthesia or hypoesthesia.2,4,6,7
Treatment with acyclovir
Oral acyclovir (2 g/day in 3 doses for 10 days) taken during the prodromal stage of recurrent HSV-2 herpetic whitlow has been shown to reduce the duration of symptoms from 10.1 to 3.7 days.8 Prophylactic use of oral acyclovir (200 mg, 4 times daily for up to 2 years) has been shown to be effective in suppressing recurrent HSV infection of nongenital skin.9
A good outcome
Our patient was given a 10-day course of acyclovir 400 mg orally 3 times daily and cefadroxil 500 mg orally twice daily (for superimposed bacterial infection). The lesions had completely disappeared upon follow-up 2 weeks later.
CORRESPONDENCE
Ossama Abbas, MD, American University of Beirut Medical Center, Riad El Solh/Beirut, Lebanon; [email protected]
The parents of an 8-year-old boy brought their son to our clinic because they were worried about the recurrent painful lesions on the pinkie, ring, and middle fingers of his right hand (FIGURE 1A and 1B). The lesions, which had recurred monthly for the past 3 years, typically lasted a few days and then spontaneously resolved.
WHAT IS YOUR DIAGNOSIS?
HOW WOULD YOU TREAT THIS PATIENT?
The diagnosis: Herpetic whitlow
Herpetic whitlow, or herpes simplex virus (HSV) infection of the hand, was first reported by Adamson in 1909.1 Herpes infection of the hand classically has a bimodal age distribution. It may be seen in children younger than 10 years of age or in adults between 20 and 30 years of age.2 In children, it is caused almost exclusively by HSV-1, whereas in adults it can be caused by HSV-1 or HSV-2.2,3
HSV infection of the hand classically occurs as a result of autoinoculation following herpetic gingivostomatitis. After inoculation, the virus has an incubation period of 2 to 20 days before vesicles appear.4 The appearance of the lesions is associated with intense throbbing pain. Fever and systemic symptoms are rare.
What you’ll see. Patients with herpetic whitlow will develop a single vesicle or cluster of vesicles on a single digit a few days after their skin has been irritated or exposed to minor trauma.2,4 Vesicles are typically clear in color and have an erythematous base. However, they are often superinfected with bacteria and may exhibit signs of impetiginization. The most common location of the vesicles is on the terminal phalanx of the thumb, index, or middle finger.3
Differential includes dactylitis
Painful fingers may also be suggestive of dactylitis.
Blistering distal dactylitis, mostly caused by group A β-hemolytic streptococci, is a bacterial infection that manifests as a tense bullae over the anterior fat pad of the volar aspect of the distal part of a single finger (or rarely a toe); diagnosis is usually confirmed by culture.5
Sickle cell dactylitis, or hand-foot syndrome, is caused by localized bone marrow infarction of the carpal and tarsal bones and phalanges. Patients will complain of a sudden onset of warm, tender global swelling of the hands and/or feet that is occasionally accompanied by fever and leucocytosis.5
Spondyloarthritis dactylitis, or a “sausage-like” digit, is usually caused by flexor tenosynovitis and presents as diffuse painful swelling of the fingers and toes, mainly over the flexor tendons.5
Making the diagnosis
The diagnosis of herpetic whitlow is clinical. If the diagnosis is unclear, diagnostic tests can include viral culture, serum antibody titers, a Tzanck smear, lesion specimen antigen testing, or histopathologic examinations. In this case, swab cultures revealed moderate growth of group A b-hemolytic streptococci. Fungal smears and cultures were negative. Histopathology revealed intraepidermal vesiculation with ballooning and reticular degeneration and cytopathic changes of herpetic infection.
The natural history of the untreated, uncomplicated herpetic whitlow is complete clearance within 2 to 3 weeks.4 Rare complications include systemic viremia, ocular infection, nail dystrophy, nail loss, scarring, and localized hyperesthesia or hypoesthesia.2,4,6,7
Treatment with acyclovir
Oral acyclovir (2 g/day in 3 doses for 10 days) taken during the prodromal stage of recurrent HSV-2 herpetic whitlow has been shown to reduce the duration of symptoms from 10.1 to 3.7 days.8 Prophylactic use of oral acyclovir (200 mg, 4 times daily for up to 2 years) has been shown to be effective in suppressing recurrent HSV infection of nongenital skin.9
A good outcome
Our patient was given a 10-day course of acyclovir 400 mg orally 3 times daily and cefadroxil 500 mg orally twice daily (for superimposed bacterial infection). The lesions had completely disappeared upon follow-up 2 weeks later.
CORRESPONDENCE
Ossama Abbas, MD, American University of Beirut Medical Center, Riad El Solh/Beirut, Lebanon; [email protected]
1. Adamson HG. Herpes febrilis attacking the fingers. Br J Dermatol. 1909;21:323-324.
2. Feder HM Jr, Long SS. Herpetic whitlow. Epidemiology, clinical characteristics, diagnosis, and treatment. Am J Dis Child. 1983;137:861-863.
3. Gill MJ, Arlette J, Tyrrell DL, et al. Herpes simplex virus infection of the hand. Clinical features and management. Am J Med. 1988;85:53-56.
4. Haedicke GJ, Grossman JA, Fisher AE. Herpetic whitlow of the digits. J Hand Surg Br. 1989;14:443-446.
5. Olivieri I, Scarano E, Padula A, et al. Dactylitis, a term for different digit diseases. Scand J Rheumatol. 2006;35:333-340.
6. Stern H, Elek SD, Millar DM, et al. Herpetic whitlow, a form of cross-infection in hospitals. Lancet. 1959;2:871-874.
7. Fowler JR. Viral infections. Hand Clin. 1989;5:613-627.
8. Gill MJ, Bryant HE. Oral acyclovir therapy of recurrent herpes simplex virus type 2 infection of the hand. Anitmicrob Agents Chemother. 1991;35:382-383.
9. Rubright JH, Shafritz AB. The herpetic whitlow. J Hand Surg Am. 2011;36:340-342.
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6. Stern H, Elek SD, Millar DM, et al. Herpetic whitlow, a form of cross-infection in hospitals. Lancet. 1959;2:871-874.
7. Fowler JR. Viral infections. Hand Clin. 1989;5:613-627.
8. Gill MJ, Bryant HE. Oral acyclovir therapy of recurrent herpes simplex virus type 2 infection of the hand. Anitmicrob Agents Chemother. 1991;35:382-383.
9. Rubright JH, Shafritz AB. The herpetic whitlow. J Hand Surg Am. 2011;36:340-342.
