March 2019 - Question 1

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Correct Answer: B 
 
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The ultrasound finding of a hyperechoic protrusion is suggestive of a gallbladder polyp. These polyps can have malignant potential and should be monitored or referred for surgical management depending on their size. There is consensus that polyps larger than 10 mm should be referred for cholecystectomy. There is some debate about whether polyps greater than 6 mm should also be referred for surgery or whether they can be surveyed. For gallbladder polyps less than 6 mm, surveillance with ultrasound in 6-12 months is the recommended surveillance strategy. 
 
Reference 
1. Gallahan WC, Conway JD. Diagnosis and management of gallbladder polyps. Gastroenterol Clin North Am. 2010;39(2):359-67.

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Correct Answer: B 
 
Rationale 
The ultrasound finding of a hyperechoic protrusion is suggestive of a gallbladder polyp. These polyps can have malignant potential and should be monitored or referred for surgical management depending on their size. There is consensus that polyps larger than 10 mm should be referred for cholecystectomy. There is some debate about whether polyps greater than 6 mm should also be referred for surgery or whether they can be surveyed. For gallbladder polyps less than 6 mm, surveillance with ultrasound in 6-12 months is the recommended surveillance strategy. 
 
Reference 
1. Gallahan WC, Conway JD. Diagnosis and management of gallbladder polyps. Gastroenterol Clin North Am. 2010;39(2):359-67.

Correct Answer: B 
 
Rationale 
The ultrasound finding of a hyperechoic protrusion is suggestive of a gallbladder polyp. These polyps can have malignant potential and should be monitored or referred for surgical management depending on their size. There is consensus that polyps larger than 10 mm should be referred for cholecystectomy. There is some debate about whether polyps greater than 6 mm should also be referred for surgery or whether they can be surveyed. For gallbladder polyps less than 6 mm, surveillance with ultrasound in 6-12 months is the recommended surveillance strategy. 
 
Reference 
1. Gallahan WC, Conway JD. Diagnosis and management of gallbladder polyps. Gastroenterol Clin North Am. 2010;39(2):359-67.

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A 63-year-old woman undergoes a right upper quadrant ultrasound for intermittent epigastric pain. A 5-mm fixed hyperechoic protrusion in the gallbladder is identified, but there are no gallstones or wall thickening. Upper endoscopy shows moderate gastritis. Biopsies reveal active H. pylori gastritis. She is treated with triple therapy and reports complete resolution of her symptoms. 
 
What is the best next step in management?

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Erythematous Periumbilical Papules and Plaques

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The Diagnosis: Metastatic Cancer

Further workup of patient 1 revealed an alkaline phosphatase level of 743 U/L (reference range, 30–120 U/L), total bilirubin level of 8.5 mg/dL (reference range, 0.3–1.2 mg/dL), and a white blood cell count of 14,000/μL (reference range, 4500–11,000/μL). Computed tomography of the abdomen and pelvis demonstrated cancer of unknown primary site that had metastasized to the colon, liver, and lungs. There was suspicion for potential colon cancer as the primary disease; however, based on the cutaneous findings, a skin biopsy was performed to confirm the diagnosis. Histology and immunohistochemistry revealed adenocarcinoma tumor cells positive for CDX2 (caudal type homeobox 2) and cytokeratin (CK) 7 with a subset positive for CK-20. The cells were negative for estrogen receptor, progesterone receptor, mammaglobin, gross cystic disease fluid protein, and GATA3 (GATA binding protein 3). Immunohistochemistry was most consistent with pancreatic cancer. During palliative percutaneous transhepatic biliary drainage placement, a liver biopsy confirmed the skin biopsy results.

Further workup of patient 2 revealed a white blood cell count of 13,000/μL (reference range, 4500–11,000/μL). Computed tomography of the chest, abdomen, and pelvis revealed metastatic disease to the lungs with a suspicion for colon cancer as the primary site. Biopsy of the skin lesion revealed a mucin-producing adenocarcinoma, and immunohistochemistry was positive for keratin (AE1/AE3), CK-20, and CDX2, consistent with metastatic colon carcinoma. Immunohistochemistry of the biopsied skin lesion was nonreactive for CK-7. The patient had a colonoscopy that revealed a fungating, partially obstructing, circumferential large mass in the ascending colon.

Metastasis to the skin from visceral malignancies is not uncommon and may represent the first evidence of widespread disease, particularly in breast cancer or mucosal cancers of the head and neck.1 Cutaneous metastasis of colon cancer is uncommon and cutaneous metastasis of pancreatic cancer is rare. Furthermore, nonumbilical sites are much more common than umbilical sites for cutaneous metastatic disease.2 Pancreatic cancer is estimated to be the origin of a cutaneous umbilical metastasis, frequently termed Sister Mary Joseph nodule, in 7% to 9% of cases; colon cancer is estimated to account for 13% to 15% of cases.3 Sister Mary Joseph nodule or sign refers to a nodule often bulging into the umbilicus, signifying metastasis from a
malignant cancer.

In a study of cutaneous metastases, 10% (42/420) of patients with metastatic disease had cutaneous metastasis; 0.48% (2/420) were due to pancreatic cancer and 4.3% (18/420) were due to colon cancer.4 In another review, 63 cases of cutaneous metastasis of pancreatic cancer were found, 43 of which were nonumbilical.

On immunohistochemistry, CK-7 positivity is highly specific for pancreatic cancer.2 Cytokeratin 7 often is used in conjunction with CK-20 to differentiate various types of glandular tumors. CDX2 is a highly sensitive and specific marker for adenocarcinomas of intestinal origin.5 The negative estrogen receptor, progesterone receptor, mammaglobin, gross cystic disease fluid protein, and GATA3 stains are useful in excluding breast cancer (patient 1 had history of breast cancer).

When cutaneous involvement is present in pancreatic cancer, the disease usually is widespread. Multiple studies have reported involvement of other organs with cutaneous metastasis at rates of 88.9%,6 90.3%,7 and 93.5%.2 However, early recognition of metastatic cancerous lesions can lead to earlier diagnosis and earlier palliative treatment, perhaps prolonging median survival time in patients. In a review of 63 patients with cutaneous metastatic pancreatic cancer, the authors found a median survival time of 5 months, with surgery, chemotherapy, radiation therapy, or a combination helping to improve survival time from a median of 3.0 to 8.3 months.2

The location of lesions and duration of disease in both patients was atypical for arthropod assault. Acyclovir-resistant herpes zoster rarely is reported outside of human immunodeficiency patients; in addition, there was a lack of clear dermatomal distribution. Although cutaneous Crohn disease can manifest as pink papules, it is rare and unlikely as a presenting symptom. Cutaneous sarcoidosis can take many different skin manifestations, and patients can have cutaneous involvement without systemic manifestation. In both patients, medical history was more indicative of metastatic cancer than the other options in the differential diagnosis.

Cutaneous metastasis from colon cancer and pancreatic cancer is rare, and the prognosis is poor in these cases; however, in the appropriate clinical scenario, especially in a patient with a history of cancer, sinister etiologies should be considered for firm red papules of the umbilicus. Skin biopsy coupled with immunohistochemical staining can assist in identifying the primary malignancy.

References
  1.   Schwartz RA. Cutaneous metastatic disease. J Am Acad Dermatol. 1995;33:161-165. 
  2. Zhou HY, Wang XB, Gao F, et al. Cutaneous metastasis from pancreatic cancer: a case report and systematic review of the literature [published online October 10, 2014]. Oncol Lett. 2014;8:2654-2660. 
  3. Galvañ VG. Sister Mary Joseph's nodule. Ann Intern Med. 1998;128:410. 
  4.  Lookingbill DP, Spangler N, Helm KF. Cutaneous metastases in patients with metastatic carcinoma: a retrospective study of 4020 patients. J Am Acad Dermatol. 1993;29:228-236. 
  5. Werling RW, Yaziji H, Bacchi CE, et al. CDX2, a highly sensitive and specific marker of adenocarcinomas of intestinal origin: an immnohistochemical survey of 476 primary and metastatic carcinomas. Am J Surg Pathol. 2003;27:303-310. 
  6. Takeuchi H, Kawano T, Toda T, et al. Cutaneous metastasis from pancreatic adenocarcinoma: a case report and a review of the literature. Hepatogastroenterology. 2003;50:275-277. 
  7. Horino K, Hiraoka T, Kanemitsu K, et al. Subcutaneous metastases after curative resection for pancreatic carcinoma: a case report and review of the literature. Pancreas. 1999;19:406-408. 
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Dr. Karmouta is from Harvard Medical School, Boston, Massachusetts. Dr. Mikailov is from the Department of Dermatology, Beth Israel Deaconess Medical Center, Boston.

The authors report no conflict of interest.

Correspondence: Ryan Karmouta, MD, MBA, Harvard Medical School, 25 Shattuck St, Boston, MA 02215 ([email protected]).

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Dr. Karmouta is from Harvard Medical School, Boston, Massachusetts. Dr. Mikailov is from the Department of Dermatology, Beth Israel Deaconess Medical Center, Boston.

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Correspondence: Ryan Karmouta, MD, MBA, Harvard Medical School, 25 Shattuck St, Boston, MA 02215 ([email protected]).

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Dr. Karmouta is from Harvard Medical School, Boston, Massachusetts. Dr. Mikailov is from the Department of Dermatology, Beth Israel Deaconess Medical Center, Boston.

The authors report no conflict of interest.

Correspondence: Ryan Karmouta, MD, MBA, Harvard Medical School, 25 Shattuck St, Boston, MA 02215 ([email protected]).

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The Diagnosis: Metastatic Cancer

Further workup of patient 1 revealed an alkaline phosphatase level of 743 U/L (reference range, 30–120 U/L), total bilirubin level of 8.5 mg/dL (reference range, 0.3–1.2 mg/dL), and a white blood cell count of 14,000/μL (reference range, 4500–11,000/μL). Computed tomography of the abdomen and pelvis demonstrated cancer of unknown primary site that had metastasized to the colon, liver, and lungs. There was suspicion for potential colon cancer as the primary disease; however, based on the cutaneous findings, a skin biopsy was performed to confirm the diagnosis. Histology and immunohistochemistry revealed adenocarcinoma tumor cells positive for CDX2 (caudal type homeobox 2) and cytokeratin (CK) 7 with a subset positive for CK-20. The cells were negative for estrogen receptor, progesterone receptor, mammaglobin, gross cystic disease fluid protein, and GATA3 (GATA binding protein 3). Immunohistochemistry was most consistent with pancreatic cancer. During palliative percutaneous transhepatic biliary drainage placement, a liver biopsy confirmed the skin biopsy results.

Further workup of patient 2 revealed a white blood cell count of 13,000/μL (reference range, 4500–11,000/μL). Computed tomography of the chest, abdomen, and pelvis revealed metastatic disease to the lungs with a suspicion for colon cancer as the primary site. Biopsy of the skin lesion revealed a mucin-producing adenocarcinoma, and immunohistochemistry was positive for keratin (AE1/AE3), CK-20, and CDX2, consistent with metastatic colon carcinoma. Immunohistochemistry of the biopsied skin lesion was nonreactive for CK-7. The patient had a colonoscopy that revealed a fungating, partially obstructing, circumferential large mass in the ascending colon.

Metastasis to the skin from visceral malignancies is not uncommon and may represent the first evidence of widespread disease, particularly in breast cancer or mucosal cancers of the head and neck.1 Cutaneous metastasis of colon cancer is uncommon and cutaneous metastasis of pancreatic cancer is rare. Furthermore, nonumbilical sites are much more common than umbilical sites for cutaneous metastatic disease.2 Pancreatic cancer is estimated to be the origin of a cutaneous umbilical metastasis, frequently termed Sister Mary Joseph nodule, in 7% to 9% of cases; colon cancer is estimated to account for 13% to 15% of cases.3 Sister Mary Joseph nodule or sign refers to a nodule often bulging into the umbilicus, signifying metastasis from a
malignant cancer.

In a study of cutaneous metastases, 10% (42/420) of patients with metastatic disease had cutaneous metastasis; 0.48% (2/420) were due to pancreatic cancer and 4.3% (18/420) were due to colon cancer.4 In another review, 63 cases of cutaneous metastasis of pancreatic cancer were found, 43 of which were nonumbilical.

On immunohistochemistry, CK-7 positivity is highly specific for pancreatic cancer.2 Cytokeratin 7 often is used in conjunction with CK-20 to differentiate various types of glandular tumors. CDX2 is a highly sensitive and specific marker for adenocarcinomas of intestinal origin.5 The negative estrogen receptor, progesterone receptor, mammaglobin, gross cystic disease fluid protein, and GATA3 stains are useful in excluding breast cancer (patient 1 had history of breast cancer).

When cutaneous involvement is present in pancreatic cancer, the disease usually is widespread. Multiple studies have reported involvement of other organs with cutaneous metastasis at rates of 88.9%,6 90.3%,7 and 93.5%.2 However, early recognition of metastatic cancerous lesions can lead to earlier diagnosis and earlier palliative treatment, perhaps prolonging median survival time in patients. In a review of 63 patients with cutaneous metastatic pancreatic cancer, the authors found a median survival time of 5 months, with surgery, chemotherapy, radiation therapy, or a combination helping to improve survival time from a median of 3.0 to 8.3 months.2

The location of lesions and duration of disease in both patients was atypical for arthropod assault. Acyclovir-resistant herpes zoster rarely is reported outside of human immunodeficiency patients; in addition, there was a lack of clear dermatomal distribution. Although cutaneous Crohn disease can manifest as pink papules, it is rare and unlikely as a presenting symptom. Cutaneous sarcoidosis can take many different skin manifestations, and patients can have cutaneous involvement without systemic manifestation. In both patients, medical history was more indicative of metastatic cancer than the other options in the differential diagnosis.

Cutaneous metastasis from colon cancer and pancreatic cancer is rare, and the prognosis is poor in these cases; however, in the appropriate clinical scenario, especially in a patient with a history of cancer, sinister etiologies should be considered for firm red papules of the umbilicus. Skin biopsy coupled with immunohistochemical staining can assist in identifying the primary malignancy.

The Diagnosis: Metastatic Cancer

Further workup of patient 1 revealed an alkaline phosphatase level of 743 U/L (reference range, 30–120 U/L), total bilirubin level of 8.5 mg/dL (reference range, 0.3–1.2 mg/dL), and a white blood cell count of 14,000/μL (reference range, 4500–11,000/μL). Computed tomography of the abdomen and pelvis demonstrated cancer of unknown primary site that had metastasized to the colon, liver, and lungs. There was suspicion for potential colon cancer as the primary disease; however, based on the cutaneous findings, a skin biopsy was performed to confirm the diagnosis. Histology and immunohistochemistry revealed adenocarcinoma tumor cells positive for CDX2 (caudal type homeobox 2) and cytokeratin (CK) 7 with a subset positive for CK-20. The cells were negative for estrogen receptor, progesterone receptor, mammaglobin, gross cystic disease fluid protein, and GATA3 (GATA binding protein 3). Immunohistochemistry was most consistent with pancreatic cancer. During palliative percutaneous transhepatic biliary drainage placement, a liver biopsy confirmed the skin biopsy results.

Further workup of patient 2 revealed a white blood cell count of 13,000/μL (reference range, 4500–11,000/μL). Computed tomography of the chest, abdomen, and pelvis revealed metastatic disease to the lungs with a suspicion for colon cancer as the primary site. Biopsy of the skin lesion revealed a mucin-producing adenocarcinoma, and immunohistochemistry was positive for keratin (AE1/AE3), CK-20, and CDX2, consistent with metastatic colon carcinoma. Immunohistochemistry of the biopsied skin lesion was nonreactive for CK-7. The patient had a colonoscopy that revealed a fungating, partially obstructing, circumferential large mass in the ascending colon.

Metastasis to the skin from visceral malignancies is not uncommon and may represent the first evidence of widespread disease, particularly in breast cancer or mucosal cancers of the head and neck.1 Cutaneous metastasis of colon cancer is uncommon and cutaneous metastasis of pancreatic cancer is rare. Furthermore, nonumbilical sites are much more common than umbilical sites for cutaneous metastatic disease.2 Pancreatic cancer is estimated to be the origin of a cutaneous umbilical metastasis, frequently termed Sister Mary Joseph nodule, in 7% to 9% of cases; colon cancer is estimated to account for 13% to 15% of cases.3 Sister Mary Joseph nodule or sign refers to a nodule often bulging into the umbilicus, signifying metastasis from a
malignant cancer.

In a study of cutaneous metastases, 10% (42/420) of patients with metastatic disease had cutaneous metastasis; 0.48% (2/420) were due to pancreatic cancer and 4.3% (18/420) were due to colon cancer.4 In another review, 63 cases of cutaneous metastasis of pancreatic cancer were found, 43 of which were nonumbilical.

On immunohistochemistry, CK-7 positivity is highly specific for pancreatic cancer.2 Cytokeratin 7 often is used in conjunction with CK-20 to differentiate various types of glandular tumors. CDX2 is a highly sensitive and specific marker for adenocarcinomas of intestinal origin.5 The negative estrogen receptor, progesterone receptor, mammaglobin, gross cystic disease fluid protein, and GATA3 stains are useful in excluding breast cancer (patient 1 had history of breast cancer).

When cutaneous involvement is present in pancreatic cancer, the disease usually is widespread. Multiple studies have reported involvement of other organs with cutaneous metastasis at rates of 88.9%,6 90.3%,7 and 93.5%.2 However, early recognition of metastatic cancerous lesions can lead to earlier diagnosis and earlier palliative treatment, perhaps prolonging median survival time in patients. In a review of 63 patients with cutaneous metastatic pancreatic cancer, the authors found a median survival time of 5 months, with surgery, chemotherapy, radiation therapy, or a combination helping to improve survival time from a median of 3.0 to 8.3 months.2

The location of lesions and duration of disease in both patients was atypical for arthropod assault. Acyclovir-resistant herpes zoster rarely is reported outside of human immunodeficiency patients; in addition, there was a lack of clear dermatomal distribution. Although cutaneous Crohn disease can manifest as pink papules, it is rare and unlikely as a presenting symptom. Cutaneous sarcoidosis can take many different skin manifestations, and patients can have cutaneous involvement without systemic manifestation. In both patients, medical history was more indicative of metastatic cancer than the other options in the differential diagnosis.

Cutaneous metastasis from colon cancer and pancreatic cancer is rare, and the prognosis is poor in these cases; however, in the appropriate clinical scenario, especially in a patient with a history of cancer, sinister etiologies should be considered for firm red papules of the umbilicus. Skin biopsy coupled with immunohistochemical staining can assist in identifying the primary malignancy.

References
  1.   Schwartz RA. Cutaneous metastatic disease. J Am Acad Dermatol. 1995;33:161-165. 
  2. Zhou HY, Wang XB, Gao F, et al. Cutaneous metastasis from pancreatic cancer: a case report and systematic review of the literature [published online October 10, 2014]. Oncol Lett. 2014;8:2654-2660. 
  3. Galvañ VG. Sister Mary Joseph's nodule. Ann Intern Med. 1998;128:410. 
  4.  Lookingbill DP, Spangler N, Helm KF. Cutaneous metastases in patients with metastatic carcinoma: a retrospective study of 4020 patients. J Am Acad Dermatol. 1993;29:228-236. 
  5. Werling RW, Yaziji H, Bacchi CE, et al. CDX2, a highly sensitive and specific marker of adenocarcinomas of intestinal origin: an immnohistochemical survey of 476 primary and metastatic carcinomas. Am J Surg Pathol. 2003;27:303-310. 
  6. Takeuchi H, Kawano T, Toda T, et al. Cutaneous metastasis from pancreatic adenocarcinoma: a case report and a review of the literature. Hepatogastroenterology. 2003;50:275-277. 
  7. Horino K, Hiraoka T, Kanemitsu K, et al. Subcutaneous metastases after curative resection for pancreatic carcinoma: a case report and review of the literature. Pancreas. 1999;19:406-408. 
References
  1.   Schwartz RA. Cutaneous metastatic disease. J Am Acad Dermatol. 1995;33:161-165. 
  2. Zhou HY, Wang XB, Gao F, et al. Cutaneous metastasis from pancreatic cancer: a case report and systematic review of the literature [published online October 10, 2014]. Oncol Lett. 2014;8:2654-2660. 
  3. Galvañ VG. Sister Mary Joseph's nodule. Ann Intern Med. 1998;128:410. 
  4.  Lookingbill DP, Spangler N, Helm KF. Cutaneous metastases in patients with metastatic carcinoma: a retrospective study of 4020 patients. J Am Acad Dermatol. 1993;29:228-236. 
  5. Werling RW, Yaziji H, Bacchi CE, et al. CDX2, a highly sensitive and specific marker of adenocarcinomas of intestinal origin: an immnohistochemical survey of 476 primary and metastatic carcinomas. Am J Surg Pathol. 2003;27:303-310. 
  6. Takeuchi H, Kawano T, Toda T, et al. Cutaneous metastasis from pancreatic adenocarcinoma: a case report and a review of the literature. Hepatogastroenterology. 2003;50:275-277. 
  7. Horino K, Hiraoka T, Kanemitsu K, et al. Subcutaneous metastases after curative resection for pancreatic carcinoma: a case report and review of the literature. Pancreas. 1999;19:406-408. 
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Erythematous Periumbilical Papules and Plaques
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A 75-year-old woman (patient 1) with a history of localized invasive ductal breast cancer treated definitively with lumpectomy and radiation therapy more than a decade ago presented to the emergency department with jaundice, abdominal pain, weakness, and multiple periumbilical pink-red papules (top) of 2 weeks’ duration. Prior to presentation, the skin lesions did not improve with 10 days of acyclovir treatment prescribed by her primary care physician for presumed herpes zoster.

An 86-year-old man (patient 2) with chronic lymphocytic leukemia treated with ibrutinib presented to the emergency department with jaundice, abdominal pain, weakness, and multiple pink periumbilical papules (bottom) of 6 weeks’ duration. Prior to presentation, the skin lesions did not improve with 21 days of valacyclovir treatment prescribed by his oncologist for presumed herpes zoster.

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January 2019 Question 2

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A 54-year-old woman presents for management of moderately severe ileocolonic Crohn’s disease. She has a strong family history of multiple sclerosis and recently noted some tingling in her toes for which she is undergoing neurologic evaluation. She has had two small basal cell carcinomas removed from her cheek in the last year. She received the BCG vaccine as a child and had a positive PPD skin test within the last year. Laboratory evaluation reveals HBsAg negative, anti-HBs positive, and anti-HBc positive; JC virus antibody is positive.

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DDSEP quick quiz December question 2

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A 54-year-old woman presents for management of moderately severe ileocolonic Crohn’s disease. She has a strong family history of multiple sclerosis and recently noted some tingling in her toes for which she is undergoing neurologic evaluation. She has had two small basal cell carcinomas removed from her cheek in the last year. She received the BCG vaccine as a child and had a positive PPD skin test within the last year. Laboratory evaluation reveals HBsAg negative, anti-HBs positive, and anti-HBc positive; JC virus antibody is positive.

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January 2019 Question 1

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A 31-year-old man is seen for a 1-week history of epigastric pain and scleral icterus. One month earlier, he developed diarrhea and fatigue, which has continued to persist. He denies any prior medical problems, though he admits he has not seen a doctor in years. He is currently visiting family in the United States, but he resides in South Africa. His laboratory tests are as follows: total bilirubin, 3.5 mg/dL; direct bilirubin, 2.9 mg/dL; alkaline phosphatase, 720 U/L; ALT, 105 U/L; AST, 117 U/L; albumin, 2.1 g/dL; INR, 1.2; HIV viral load 450,000 copies/mL, CD4 count, 25 cells/mm3. An abdominal ultrasound shows intra- and extrahepatic ductal dilation and an ERCP shows strictured intrahepatic ducts with papillary stenosis.

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December 2018

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A 63-year-old white female presented with a 2-week history of hemorrhagic purpuric lesions and necrotic vesicles on the bilateral lower extremities.

Vasculitis is a process in which blood vessels become inflamed and necrotic. Classic small-vessel vasculitis reveals a leukocytoclastic vasculitis and most commonly presents as palpable purpura. In addition to skin, organs such as joints, kidneys, and intestines can be involved.

Henoch-Schönlein Purpura (HSP), also called anaphylactoid purpura, is a subtype of small-vessel vasculitis where immunoglobulin A (IgA) is deposited in the vessel walls. It is the most common form of vasculitis in children (usually aged 4-8 years). The incidence is higher in the winter. Some patients experience a prodrome of fever, colicky abdominal pain, and joint pain prior to the development of cutaneous symptoms. Disease in children tends to be self-limited. Adults may present with HSP as well, and often exhibit more severe disease that may become chronic with relapses and is more difficult to treat. In both children and adults, infectious causes, such as streptococcus pharyngitis, are the most common trigger. In adults, malignancy may be associated with HSP. A literature search revealed medications implicated in HSP such as antibiotics (vancomycin, penicillin, cephalosporins, clarithromycin), ACE inhibitors, and nonsteroidal anti-inflammatories. Many cases of HSP are idiopathic.

Patients present with erythematous macules that progress to purpura on the extremities. Lesions may be vesicular or bullous and may become necrotic and ulcerate. Arthralgias, often of lower-extremity joints, may be present. Abdominal pain and renal disease may occur in both children and adults. Adults are more likely to develop chronic kidney disease and must be followed carefully with serial blood work and urinalysis to evaluate for hematuria and proteinuria. Severe abdominal pain is an emergency as intussusception may occur.

Dr. Donna Bilu Martin

Histologically, leukocytoclastic vasculitis of small vessels is present. On direct immunofluorescence of perilesional skin, IgA, C3, and fibrin deposits can be seen. Serum IgA is unreliable and may be seen in healthy adults as well.

Treatment is generally supportive as the disease is self-limited. The use of corticosteroids is controversial. This may be effective for joint inflammation, abdominal disease, active nephritis, and ulcerated skin lesions, but doesn’t prevent the recurrence of skin lesions. Dapsone or colchicine can be used for resistant cutaneous lesions. In severe cases, intravenous immunoglobulin may be warranted.

This case and photo were submitted by Dr. Bilu Martin.

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at edermatologynews.com. To submit a case for possible publication, send an email to [email protected].

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Vasculitis is a process in which blood vessels become inflamed and necrotic. Classic small-vessel vasculitis reveals a leukocytoclastic vasculitis and most commonly presents as palpable purpura. In addition to skin, organs such as joints, kidneys, and intestines can be involved.

Henoch-Schönlein Purpura (HSP), also called anaphylactoid purpura, is a subtype of small-vessel vasculitis where immunoglobulin A (IgA) is deposited in the vessel walls. It is the most common form of vasculitis in children (usually aged 4-8 years). The incidence is higher in the winter. Some patients experience a prodrome of fever, colicky abdominal pain, and joint pain prior to the development of cutaneous symptoms. Disease in children tends to be self-limited. Adults may present with HSP as well, and often exhibit more severe disease that may become chronic with relapses and is more difficult to treat. In both children and adults, infectious causes, such as streptococcus pharyngitis, are the most common trigger. In adults, malignancy may be associated with HSP. A literature search revealed medications implicated in HSP such as antibiotics (vancomycin, penicillin, cephalosporins, clarithromycin), ACE inhibitors, and nonsteroidal anti-inflammatories. Many cases of HSP are idiopathic.

Patients present with erythematous macules that progress to purpura on the extremities. Lesions may be vesicular or bullous and may become necrotic and ulcerate. Arthralgias, often of lower-extremity joints, may be present. Abdominal pain and renal disease may occur in both children and adults. Adults are more likely to develop chronic kidney disease and must be followed carefully with serial blood work and urinalysis to evaluate for hematuria and proteinuria. Severe abdominal pain is an emergency as intussusception may occur.

Dr. Donna Bilu Martin

Histologically, leukocytoclastic vasculitis of small vessels is present. On direct immunofluorescence of perilesional skin, IgA, C3, and fibrin deposits can be seen. Serum IgA is unreliable and may be seen in healthy adults as well.

Treatment is generally supportive as the disease is self-limited. The use of corticosteroids is controversial. This may be effective for joint inflammation, abdominal disease, active nephritis, and ulcerated skin lesions, but doesn’t prevent the recurrence of skin lesions. Dapsone or colchicine can be used for resistant cutaneous lesions. In severe cases, intravenous immunoglobulin may be warranted.

This case and photo were submitted by Dr. Bilu Martin.

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at edermatologynews.com. To submit a case for possible publication, send an email to [email protected].

Vasculitis is a process in which blood vessels become inflamed and necrotic. Classic small-vessel vasculitis reveals a leukocytoclastic vasculitis and most commonly presents as palpable purpura. In addition to skin, organs such as joints, kidneys, and intestines can be involved.

Henoch-Schönlein Purpura (HSP), also called anaphylactoid purpura, is a subtype of small-vessel vasculitis where immunoglobulin A (IgA) is deposited in the vessel walls. It is the most common form of vasculitis in children (usually aged 4-8 years). The incidence is higher in the winter. Some patients experience a prodrome of fever, colicky abdominal pain, and joint pain prior to the development of cutaneous symptoms. Disease in children tends to be self-limited. Adults may present with HSP as well, and often exhibit more severe disease that may become chronic with relapses and is more difficult to treat. In both children and adults, infectious causes, such as streptococcus pharyngitis, are the most common trigger. In adults, malignancy may be associated with HSP. A literature search revealed medications implicated in HSP such as antibiotics (vancomycin, penicillin, cephalosporins, clarithromycin), ACE inhibitors, and nonsteroidal anti-inflammatories. Many cases of HSP are idiopathic.

Patients present with erythematous macules that progress to purpura on the extremities. Lesions may be vesicular or bullous and may become necrotic and ulcerate. Arthralgias, often of lower-extremity joints, may be present. Abdominal pain and renal disease may occur in both children and adults. Adults are more likely to develop chronic kidney disease and must be followed carefully with serial blood work and urinalysis to evaluate for hematuria and proteinuria. Severe abdominal pain is an emergency as intussusception may occur.

Dr. Donna Bilu Martin

Histologically, leukocytoclastic vasculitis of small vessels is present. On direct immunofluorescence of perilesional skin, IgA, C3, and fibrin deposits can be seen. Serum IgA is unreliable and may be seen in healthy adults as well.

Treatment is generally supportive as the disease is self-limited. The use of corticosteroids is controversial. This may be effective for joint inflammation, abdominal disease, active nephritis, and ulcerated skin lesions, but doesn’t prevent the recurrence of skin lesions. Dapsone or colchicine can be used for resistant cutaneous lesions. In severe cases, intravenous immunoglobulin may be warranted.

This case and photo were submitted by Dr. Bilu Martin.

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at edermatologynews.com. To submit a case for possible publication, send an email to [email protected].

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A 63-year-old white female presented with a 2-week history of hemorrhagic purpuric lesions and necrotic vesicles on the bilateral lower extremities.
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A 63-year-old white female presented with a 2-week history of hemorrhagic purpuric lesions and necrotic vesicles on the bilateral lower extremities.
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A 63-year-old white female presented with a 2-week history of hemorrhagic purpuric lesions and necrotic vesicles on the bilateral lower extremities.

Nearly 1 year prior to presentation, the patient underwent surgical resection for lung cancer. The patient also complained of joint swelling and pain in her ankles. She denied abdominal pain. She denied recent illness, including sore throat and upper respiratory infection. Skin biopsies were performed, including for direct immunofluorescence.

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