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Confluent and reticulate papillomatosis of Gougerot and Carteaud: A review of 7 patients in National Skin Centre, Singapore
Cutaneous collagenous vasculopathy in an 18-year-old white female
Confluent and reticulate papillomatosis of Gougerot and Carteaud: A review of 7 patients in National Skin Centre, Singapore
(Poster reference number 4741)
Gavin Chun Wei ONG, MBBS, National Skin Centre, Singapore; Wei Sheng Chong, MBBS, National Skin Centre, Singapore
Elizabeth Chase, MD, UC Davis Medical Center, Sacramento, CA, United States; Anna Asher, MD, Belle Meade Medical, Flowood, MS, United States; Jennifer Schulmeier, MD, Dermatopathology Associates, Jackson, MS, United States
(Poster reference number 5385)
Background: Confluent and reticulate papillomatosis of Gougerot and Carteaud (CRP) is an uncommon skin disorder whose pathogenesis is poorly understood. It was previously thought to be a result of abnormal host response to Malassezia furfur. Past descriptions of CRP were limited to case reports and small case series. Diagnostic criteria were previously proposed by Davis et al (2005). This study aims to characterize the clinical and pathologic findings of CRP in our local setting and in relation to the proposed diagnostic criteria. Methods: We retrospectively viewed the clinical presentation, histologic findings, and response to treatment of patients presenting to the National Skin Centre, Singapore with CRP. Results: There were 7 patients diagnosed with CRP with concurrent histologic examination between 1985-2011. The majority of the patients were young, with a mean age of onset of 20.4 years (range, 16-32). Five patients (71.4%) were male. At presentation, the mean duration of presentation was 1.5 years (range, 6 months-3 years). The majority presented with reticulate, hyperpigmented, sometimes scaly macules affecting the neck, back, cubital fossae, and upper and lower limbs. The most frequent initial diagnoses were CRP, pityriasis versicolor, and acanthosis nigricans. Four of 7 who underwent fungal scraping were tested negative. Treatments given included minocycline, doxycycline, erythromycin, topical and oral ketoconazole, topical retinoids and salicylic acid. The majority of patients had clinical improvement only when given oral antibiotics. When the proposed diagnostic criteria were applied, 5 patients (71.4%) fulfilled at least 4 out of 5 of the diagnostic criteria. Conclusion: CRP is an uncommon skin disorder occurring mostly in young adults and is often misdiagnosed, resulting in treatment delay. The absence of positive fungal scrap and the apparent lack of response to antifungal treatment, seems to imply that fungi are not involved in the pathogenesis of this condition. The proposed diagnostic criteria correlated well in our patients; it could be used in future to aid diagnosis and avoid invasive procedure such as skin biopsy.
Cutaneous collagenous vasculopathy (CCV) is an exceedingly rare diagnosis with 8 total cases described in world literature. Initially believed to only affect middle-aged men, a recent report described 2 female patients affected by the disorder. We present here a case of an 18-year-old white woman with no medical history taking no medications who presented to our clinic with a several month history of an asymptomatic rash on her chest, abdomen, and back. The patient denied urtication of the lesions. The patient had had a previous biopsy which was consistent with lymphocytic perivascular dermatitis. On physical examination, numerous erythematous, blanchable patches over the chest, abdomen, back, and left shoulder were noted as well as numerous light brown macules on face, chest, back, and extremities. Darier sign was negative. CBC, SGOT hepatitis panel, ANA, Lyme antibodies, anti-SSA, and anti-SSB antibodies were negative. ESR, ASO, and CRP were within normal limits. A rebiopsy of normal and involved tissue using normal saline for anesthesia was performed while the patient avoided antihistamines, topical steroids, and NSAIDS before biopsy. Biopsy of involved skin was consistent with a form of telangiectasia and demonstrated a mild perivascular infiltrate with the tissue lacking sufficient numbers of mast cells for diagnosis of telangiectasia macularis eruptiva perstans. However, the vessels in the involved skin specimen had markedly thickened walls with hyalinized appearance, consistent with CCV. A trial treatment of a 595-nm pulsed dye laser to the affected areas completely resolved the erythematous patches, though some lesions resurfaced a year later. To the best of our knowledge, this is the youngest patient with CCV ever reported. Commercial support: None identified.
Commercial support: None identified.
Crohn disease associated with vasculopathy—A harbinger of active bowel disease?
(Poster reference number 5284)
Paula Malhotra, MD, University of Louisville, Louisville, KY, United States; Janine Malone, MD, University of Louisville, Louisville, KY, United States; Jeffrey Callen, MD, University of Louisville, Louisville, KY, United States Background: Crohn disease (CD) is a chronic granulomatous disease of any part of the bowel. Cutaneous manifestations include pyoderma gangrenosum, erythema nodosum, an acrodermatitis-like eruption, and necrotizing vasculitis. We describe a case of a 23-year-old woman with CD who developed an acute necrotizing vasculopathy. Observation: A 23-year-old woman with a history of ulcerative colitis had worsening bloody diarrhea. Colonoscopy revealed changes that were more consistent with CD. Two days after colonoscopy, the patient’s bowel symptoms worsened and she developed painful necrotic, purpuric, stellate, and bullous plaques on her extremities, abdomen, and breasts. Biopsies revealed fibrin thrombi in dermal blood vessels with a lack of associated inflammation consistent with an occlusive vasculopathy. Blood work including tests for thrombophilic states and autoimmune diseases were negative or within normal limits. We also tested her initial serum from the outside facility for protein C/S as delayed testing can often result in false negative; however, those tests were also within normal limits. Drug screen was negative. Her only medications included azathioprine, mesalamine, and oral contraceptive pills (which she had been on for a year). She was initially treated with IV corticosteroids which appeared to halt the progression of disease and improved the activity of her inflammatory bowel disease, but she developed bowel perforation and sepsis and succumbed to her disease.
Cutaneous metastatic Crohn disease of the leg
(Poster reference number 4640)
Brooke Walls, DO, NOVA Southeastern University College of Osteopathic Medicine, Largo, FL, United States; David Judy, DO, NOVA Southeastern University College of Osteopathic Medicine, Largo, FL, United States; Eli Piatigorsky, MD, Global Pathology Services, Miami Lakes, FL, United States; Lana McKinley, DO, NOVA Southeastern University College of Osteopathic Medicine, Largo, FL, United States
Discussion: Cutaneous complications are reported to occur in 14% to 44% of patients with CD. Vasculitis is more commonly associated with CD than vasculopathy and has been associated with active phases of intestinal disease, rarely preceding intestinal symptoms. In our case, vasculopathy may have been a marker of impending bowel disease. Whether this patient’s skin lesions were related to her labile bowel disease and a manifestation of possible acute clotting factor imbalance in regards to protein C deficiency is unknown. This patient presents an interesting case of active CD associated with a vasculopathy. Perhaps her skin lesions were a harbinger of active bowel disease.
Cutaneous metastatic Crohn disease (CMCD) is a rare extraintestinal manifestation of inflammatory bowel disease characterized by cutaneous lesions at sites noncontiguous with alimentary tract. We report a case of a 49-year-old woman with an approximately 10 cm 3 12 cm, progressively enlarging, tender, purulent plaque with scattered ulcerations and underlying erythema located on her posterior calf. The medical history is positive for Crohn disease (CD) diagnosed 25 years earlier status postesmall and large bowel resections secondary to complications; however, the patient denies concurrent bowel symptoms upon presentation of the cuntaneous lesions. Histologic examination revealed ulceration accompanied by reactive hyperplasia of the epidermis and nonsuppurative granulomatous dermal inflammation. Multinucleated foreign body-type giant cells were present. CMCD is a rare manifestation of CD and often misdiagnosed. A clinical history of intestinal CD is a critical diagnostic clue as the differential diagnosis can be quite vast. Although our patient presented with quiescent CD, the clinicopathologic correlation was imperative in making the final diagnosis.
Commercial support: None identified.
Commercial support: None identified.
APRIL 2012
J AM ACAD DERMATOL
AB47
Confluent and reticulate papillomatosis of Gougerot and Carteaud: A review of 7 patients in National Skin Centre, Singapore
(Poster reference number 4741)
Gavin Chun Wei ONG, MBBS, National Skin Centre, Singapore; Wei Sheng Chong, MBBS, National Skin Centre, Singapore
Elizabeth Chase, MD, UC Davis Medical Center, Sacramento, CA, United States; Anna Asher, MD, Belle Meade Medical, Flowood, MS, United States; Jennifer Schulmeier, MD, Dermatopathology Associates, Jackson, MS, United States
(Poster reference number 5385)
Background: Confluent and reticulate papillomatosis of Gougerot and Carteaud (CRP) is an uncommon skin disorder whose pathogenesis is poorly understood. It was previously thought to be a result of abnormal host response to Malassezia furfur. Past descriptions of CRP were limited to case reports and small case series. Diagnostic criteria were previously proposed by Davis et al (2005). This study aims to characterize the clinical and pathologic findings of CRP in our local setting and in relation to the proposed diagnostic criteria. Methods: We retrospectively viewed the clinical presentation, histologic findings, and response to treatment of patients presenting to the National Skin Centre, Singapore with CRP. Results: There were 7 patients diagnosed with CRP with concurrent histologic examination between 1985-2011. The majority of the patients were young, with a mean age of onset of 20.4 years (range, 16-32). Five patients (71.4%) were male. At presentation, the mean duration of presentation was 1.5 years (range, 6 months-3 years). The majority presented with reticulate, hyperpigmented, sometimes scaly macules affecting the neck, back, cubital fossae, and upper and lower limbs. The most frequent initial diagnoses were CRP, pityriasis versicolor, and acanthosis nigricans. Four of 7 who underwent fungal scraping were tested negative. Treatments given included minocycline, doxycycline, erythromycin, topical and oral ketoconazole, topical retinoids and salicylic acid. The majority of patients had clinical improvement only when given oral antibiotics. When the proposed diagnostic criteria were applied, 5 patients (71.4%) fulfilled at least 4 out of 5 of the diagnostic criteria. Conclusion: CRP is an uncommon skin disorder occurring mostly in young adults and is often misdiagnosed, resulting in treatment delay. The absence of positive fungal scrap and the apparent lack of response to antifungal treatment, seems to imply that fungi are not involved in the pathogenesis of this condition. The proposed diagnostic criteria correlated well in our patients; it could be used in future to aid diagnosis and avoid invasive procedure such as skin biopsy.
Cutaneous collagenous vasculopathy (CCV) is an exceedingly rare diagnosis with 8 total cases described in world literature. Initially believed to only affect middle-aged men, a recent report described 2 female patients affected by the disorder. We present here a case of an 18-year-old white woman with no medical history taking no medications who presented to our clinic with a several month history of an asymptomatic rash on her chest, abdomen, and back. The patient denied urtication of the lesions. The patient had had a previous biopsy which was consistent with lymphocytic perivascular dermatitis. On physical examination, numerous erythematous, blanchable patches over the chest, abdomen, back, and left shoulder were noted as well as numerous light brown macules on face, chest, back, and extremities. Darier sign was negative. CBC, SGOT hepatitis panel, ANA, Lyme antibodies, anti-SSA, and anti-SSB antibodies were negative. ESR, ASO, and CRP were within normal limits. A rebiopsy of normal and involved tissue using normal saline for anesthesia was performed while the patient avoided antihistamines, topical steroids, and NSAIDS before biopsy. Biopsy of involved skin was consistent with a form of telangiectasia and demonstrated a mild perivascular infiltrate with the tissue lacking sufficient numbers of mast cells for diagnosis of telangiectasia macularis eruptiva perstans. However, the vessels in the involved skin specimen had markedly thickened walls with hyalinized appearance, consistent with CCV. A trial treatment of a 595-nm pulsed dye laser to the affected areas completely resolved the erythematous patches, though some lesions resurfaced a year later. To the best of our knowledge, this is the youngest patient with CCV ever reported. Commercial support: None identified.
Commercial support: None identified.
Crohn disease associated with vasculopathy—A harbinger of active bowel disease?
(Poster reference number 5284)
Paula Malhotra, MD, University of Louisville, Louisville, KY, United States; Janine Malone, MD, University of Louisville, Louisville, KY, United States; Jeffrey Callen, MD, University of Louisville, Louisville, KY, United States Background: Crohn disease (CD) is a chronic granulomatous disease of any part of the bowel. Cutaneous manifestations include pyoderma gangrenosum, erythema nodosum, an acrodermatitis-like eruption, and necrotizing vasculitis. We describe a case of a 23-year-old woman with CD who developed an acute necrotizing vasculopathy. Observation: A 23-year-old woman with a history of ulcerative colitis had worsening bloody diarrhea. Colonoscopy revealed changes that were more consistent with CD. Two days after colonoscopy, the patient’s bowel symptoms worsened and she developed painful necrotic, purpuric, stellate, and bullous plaques on her extremities, abdomen, and breasts. Biopsies revealed fibrin thrombi in dermal blood vessels with a lack of associated inflammation consistent with an occlusive vasculopathy. Blood work including tests for thrombophilic states and autoimmune diseases were negative or within normal limits. We also tested her initial serum from the outside facility for protein C/S as delayed testing can often result in false negative; however, those tests were also within normal limits. Drug screen was negative. Her only medications included azathioprine, mesalamine, and oral contraceptive pills (which she had been on for a year). She was initially treated with IV corticosteroids which appeared to halt the progression of disease and improved the activity of her inflammatory bowel disease, but she developed bowel perforation and sepsis and succumbed to her disease.
Cutaneous metastatic Crohn disease of the leg
(Poster reference number 4640)
Brooke Walls, DO, NOVA Southeastern University College of Osteopathic Medicine, Largo, FL, United States; David Judy, DO, NOVA Southeastern University College of Osteopathic Medicine, Largo, FL, United States; Eli Piatigorsky, MD, Global Pathology Services, Miami Lakes, FL, United States; Lana McKinley, DO, NOVA Southeastern University College of Osteopathic Medicine, Largo, FL, United States
Discussion: Cutaneous complications are reported to occur in 14% to 44% of patients with CD. Vasculitis is more commonly associated with CD than vasculopathy and has been associated with active phases of intestinal disease, rarely preceding intestinal symptoms. In our case, vasculopathy may have been a marker of impending bowel disease. Whether this patient’s skin lesions were related to her labile bowel disease and a manifestation of possible acute clotting factor imbalance in regards to protein C deficiency is unknown. This patient presents an interesting case of active CD associated with a vasculopathy. Perhaps her skin lesions were a harbinger of active bowel disease.
Cutaneous metastatic Crohn disease (CMCD) is a rare extraintestinal manifestation of inflammatory bowel disease characterized by cutaneous lesions at sites noncontiguous with alimentary tract. We report a case of a 49-year-old woman with an approximately 10 cm 3 12 cm, progressively enlarging, tender, purulent plaque with scattered ulcerations and underlying erythema located on her posterior calf. The medical history is positive for Crohn disease (CD) diagnosed 25 years earlier status postesmall and large bowel resections secondary to complications; however, the patient denies concurrent bowel symptoms upon presentation of the cuntaneous lesions. Histologic examination revealed ulceration accompanied by reactive hyperplasia of the epidermis and nonsuppurative granulomatous dermal inflammation. Multinucleated foreign body-type giant cells were present. CMCD is a rare manifestation of CD and often misdiagnosed. A clinical history of intestinal CD is a critical diagnostic clue as the differential diagnosis can be quite vast. Although our patient presented with quiescent CD, the clinicopathologic correlation was imperative in making the final diagnosis.
Commercial support: None identified.
Commercial support: None identified.
APRIL 2012
J AM ACAD DERMATOL
AB47