- Email: [email protected]
A case of necrobiotic xanthogranuloma treated with prednisolone
1539
832
A case of necrobiotic xanthogranuloma treated with prednisolone Chris Duhovic, Kent & Canterbury Hospital, Canterbury, United Kingdom; Emilia Duarte Williamson, Kent & Canterbury Hospital, Canterbury, United Kingdom A 62-year-old lady had skin lesions on her upper and lower eyelids removed by plastic surgeons. This was thought to be xanthelasma as she was known to have hypercholesterolemia. She was then referred to the ophthalmologists because the lesions were increasing in size and were causing a mechanical ptosis. Further skin biopsies were taken and histology showed variable numbers of histiocytes, foamy macrophages, and Touton giant cells. There were also scattered lymphocytes, eosinophils, foci of collagen degeneration and cholesterol cleft formation. These were features consistent with necrobiotic xanthogranuloma (NXG). Blood investigations showed an IgG kappa paraproteinemia. Hematologists arranged a bone marrow biopsy which showed no evidence of myeloma. It was concluded that she had monoclonal gammopathy of unknown significance (MGUS). MRI imaging of the orbits incidentally showed extensive nasal polyps which were managed conservatively. She was largely asymptomatic from these and declined a polypectomy. She received a course of prednisolone for sinusitis and incidentally a reduction in periorbital swelling and flattening of the periorbital papules and nodules was noted. Methotrexate was introduced although was stopped not long after due to deranged liver function tests. Thalidomide was also trialed but discontinued due to a lack of improvement. Prednisolone alone was continued at 20 mg daily and this dose was gradually tapered. Necrobiotic xanthogranuloma usually presents as firm yellow plaques and nodules, usually in a periorbital distribution. It was first described as a distinct entity from xanthoma in 1980 by Kossard and Winkelmann. Lymphoproliferative disorders have been reported in the literature highlighting the importance of performing a bone marrow biopsy. Systemic involvement can occur, with the most common site being the respiratory tract. The success of treatments is mixed, and these include surgical excision, intravenous immunoglobulin, and alkylating agents such as chlorambucil, melphalan and cyclophosphamide. Our patient has seen improvement of her skin lesions since she was initiated on prednisolone and is now on a maintenance dose of 5 mg on alternate days. No further treatment has been proposed at this stage, and her gammopathy is being monitored. The adjusted lifetime risk of progression of MGUS to myeloma is quoted as 11%, so any decision to proceed to more aggressive treatment for skin lesions would require careful consideration.
Acquired acrodermatitis enteropathica secondary to antihypertensive therapy Nirav Patel, MD, Mayo Clinic, Phoenix, AZ, United States; David DiCaudo, MD, Mayo Clinic, Scottsdale, AZ, United States A 94-year-old-male presented with a three-month history of a worsening desquamative, painful eruption in the perianal region. He had no history of fecal incontinence or pruritis in the area prior to appearance of the eruption. He did not have a history of using toilet wipes. On exam, the patient had large, sharply marginated ulcers with a punched out appearance. The ulcers had a yellow-brown eschar, and surrounding erythema with scaling. He also had an ulcer on the tip of the tongue, and an oval, pink patch along the vermillion border with erosion and hemorrhagic crust. Investigation was initiated with a punch biopsy for histology, viral culture, PCR for herpes simplex virus (HSV) and varicella zoster virus (VZV), and direct smear with fluorescent antibody assay for HSV and VZV. Punch biopsy demonstrated superficial ulceration with epidermal vacuolization and dyskeratotic keratinocytes involving the superficial portions of the epidermis. Methenamine silver stain was negative for fungal organisms. Immunostains for HSV 1, HSV 2, and cytomegalovirus were negative. Given the histopathology, labs for nutritional deficiency were ordered. The patient was evaluated by a nutritionist and was found to have a normal diet. He did not have a history of weight loss or diarrhea. Laboratory studies revealed a low zinc level of 0.56 mcg/mL (normal range 0.66-1.1 mcg/mL). The clinical appearance, histopathology, and low zinc level suggested a diagnosis of acquired acrodermatitis enteropathica (AAE). The patient was started on zinc supplementation at 1 mg/kg. He had rapid improvement in one week. AAE can occur at any age due to low zinc levels. Poor dietary intake, intestinal loss, and poor absorption are the most common causes. Our patient had a balanced diet with no evidence of intestinal loss or poor absorption of nutrients. Antihypertensives have been shown to cause decreased zinc levels through urinary loss. Our patient was taking both lisinopril and hydrochlorothiazide, which have been documented to cause a decrease in zinc levels. Because of his antihypertensive therapy, our patient will remain on zinc supplementation, even after complete clearance of his eruption. Commercial support: None identified.
Commercial support: None identified.
1287 A retrospective review of biopsy-proven calciphylaxis in a tertiary center in Singapore: Clinical features and prognosis in a Chinese population Yi Wei Yeo, MBBS, Singapore General Hospital, Singapore; Chay Mien Lim, Singapore General Hospital, Singapore; Hong Yi Koh, MBBS, Singapore General Hospital, Singapore; Thamotharampillai Thirumoorthy, MBBS, Singapore General Hospital, Singapore; Shiu Ming Pang, MBBS, Singapore General Hospital, Singapore; Haur Yueh Lee, MBBS, Singapore General Hospital, Singapore Background: Calciphylaxis is a small vessel obliterative vasculopathy characterized by mural calcification resulting in painful ischemic cutaneous ulceration. Mortality is high with a reported 1 year survival rate of 45.8% in Western populations. It is unclear if such high mortality is also present in other ethnic populations. We aimed to determine the epidemiologic profile, clinical features and outcomes of patients with calciphylaxis seen at an academic medical center in Singapore. Methods: A retrospective review of all biopsy-proven calciphylaxis cases seen by the Department of Dermatology, Singapore General Hospital from January 2012 to May 2014 was performed. Clinical and follow-up data were obtained from medical records. Results: A total of 16 biopsy-proven cases of calciphylaxis were identified. 14 were uremic while 2 were of nonuremic calciphylaxis. All patients were Chinese with a mean age of 57 years and a marked female preponderance of 93.8% (n ¼ 15). Of the uremic patients on dialysis, 8 (61.5%) were on hemodialysis while 4 (38.5%) were on peritoneal dialysis (mean duration ¼ 87.5 months). All patients had multiple comorbidities including diabetes mellitus, hypertension, ischemic heart disease and peripheral vascular disease. 2 patients with nonuremic calciphylaxis had systemic lupus erythematous and systemic sclerosis, respectively. In terms of clinical features, all patients presented with painful eschars and/or ulcers of the distal limbs with 2 (12.5%) also having proximal limb involvement. In addition, 8 (50%) had retiform purpura and 7 (44%) had induration suggestive of a panniculitis. 13 (81.3%) patients received intravenous sodium thiosulphate with 75% also being switched to noncalcium phosphate binders. 1 patient had hyperbaric oxygen therapy. 3 (18.8%) had surgical parathyroidectomy and 4 (25%) who were deemed medically unfit received cinacalcet instead. 4 (25%) had angioplasty and 5 (31.3%) had surgical debridement with 2 (12.5%) resulting in amputation. 13 patients were followed up beyond 1 year giving a 1- year mortality rate of 61.5% (n ¼ 8). All patients on peritoneal dialysis died by 13 months postdiagnosis.
530
Conclusion: This study shows that mortality remains high despite routine use of sodium thiosulphate and noncalcium phosphate binders. Our data also support previous studies that those on peritoneal dialysis may be associated with a poorer prognosis.
All trans-retinoic acid (ATRA)-induced Sweet syndrome in a leukemia patient Brandon Shutty, DO, Nova Southeastern University/Largo Medical Center, Largo, FL, United States; Richard Miller, DO, Nova Southeastern University/Largo Medical Center, Largo, FL, United States; David Dorton, DO, Nova Southeastern University/Largo Medical Center, Largo, FL, United States; George Gibbons, MD, Nova Southeastern University/Largo Medical Center, Largo, FL, United States; Jared Heaton, DO, Nova Southeastern University, Largo, FL, United States Sweet syndrome (SS), or acute febrile neutrophilic dermatosis, is a condition described as having painful erythematous papules and plaques along with the rapid onset of fever and leukocytosis. Generally this syndrome is idiopathic; however, associations exist with hematologic disorders, infection, connective tissue disease, or postexposure to drug therapy. All trans-retinoic acid (ATRA) is one such therapy rarely reported to be associated with the development of SS. This is exemplified by the case we present where a patient developed SS after treatment with ATRA for acute promyelocytic leukemia.
Commercial support: None identified.
Commercial support: None identified.
MAY 2015
J AM ACAD DERMATOL
AB143
832
A case of necrobiotic xanthogranuloma treated with prednisolone Chris Duhovic, Kent & Canterbury Hospital, Canterbury, United Kingdom; Emilia Duarte Williamson, Kent & Canterbury Hospital, Canterbury, United Kingdom A 62-year-old lady had skin lesions on her upper and lower eyelids removed by plastic surgeons. This was thought to be xanthelasma as she was known to have hypercholesterolemia. She was then referred to the ophthalmologists because the lesions were increasing in size and were causing a mechanical ptosis. Further skin biopsies were taken and histology showed variable numbers of histiocytes, foamy macrophages, and Touton giant cells. There were also scattered lymphocytes, eosinophils, foci of collagen degeneration and cholesterol cleft formation. These were features consistent with necrobiotic xanthogranuloma (NXG). Blood investigations showed an IgG kappa paraproteinemia. Hematologists arranged a bone marrow biopsy which showed no evidence of myeloma. It was concluded that she had monoclonal gammopathy of unknown significance (MGUS). MRI imaging of the orbits incidentally showed extensive nasal polyps which were managed conservatively. She was largely asymptomatic from these and declined a polypectomy. She received a course of prednisolone for sinusitis and incidentally a reduction in periorbital swelling and flattening of the periorbital papules and nodules was noted. Methotrexate was introduced although was stopped not long after due to deranged liver function tests. Thalidomide was also trialed but discontinued due to a lack of improvement. Prednisolone alone was continued at 20 mg daily and this dose was gradually tapered. Necrobiotic xanthogranuloma usually presents as firm yellow plaques and nodules, usually in a periorbital distribution. It was first described as a distinct entity from xanthoma in 1980 by Kossard and Winkelmann. Lymphoproliferative disorders have been reported in the literature highlighting the importance of performing a bone marrow biopsy. Systemic involvement can occur, with the most common site being the respiratory tract. The success of treatments is mixed, and these include surgical excision, intravenous immunoglobulin, and alkylating agents such as chlorambucil, melphalan and cyclophosphamide. Our patient has seen improvement of her skin lesions since she was initiated on prednisolone and is now on a maintenance dose of 5 mg on alternate days. No further treatment has been proposed at this stage, and her gammopathy is being monitored. The adjusted lifetime risk of progression of MGUS to myeloma is quoted as 11%, so any decision to proceed to more aggressive treatment for skin lesions would require careful consideration.
Acquired acrodermatitis enteropathica secondary to antihypertensive therapy Nirav Patel, MD, Mayo Clinic, Phoenix, AZ, United States; David DiCaudo, MD, Mayo Clinic, Scottsdale, AZ, United States A 94-year-old-male presented with a three-month history of a worsening desquamative, painful eruption in the perianal region. He had no history of fecal incontinence or pruritis in the area prior to appearance of the eruption. He did not have a history of using toilet wipes. On exam, the patient had large, sharply marginated ulcers with a punched out appearance. The ulcers had a yellow-brown eschar, and surrounding erythema with scaling. He also had an ulcer on the tip of the tongue, and an oval, pink patch along the vermillion border with erosion and hemorrhagic crust. Investigation was initiated with a punch biopsy for histology, viral culture, PCR for herpes simplex virus (HSV) and varicella zoster virus (VZV), and direct smear with fluorescent antibody assay for HSV and VZV. Punch biopsy demonstrated superficial ulceration with epidermal vacuolization and dyskeratotic keratinocytes involving the superficial portions of the epidermis. Methenamine silver stain was negative for fungal organisms. Immunostains for HSV 1, HSV 2, and cytomegalovirus were negative. Given the histopathology, labs for nutritional deficiency were ordered. The patient was evaluated by a nutritionist and was found to have a normal diet. He did not have a history of weight loss or diarrhea. Laboratory studies revealed a low zinc level of 0.56 mcg/mL (normal range 0.66-1.1 mcg/mL). The clinical appearance, histopathology, and low zinc level suggested a diagnosis of acquired acrodermatitis enteropathica (AAE). The patient was started on zinc supplementation at 1 mg/kg. He had rapid improvement in one week. AAE can occur at any age due to low zinc levels. Poor dietary intake, intestinal loss, and poor absorption are the most common causes. Our patient had a balanced diet with no evidence of intestinal loss or poor absorption of nutrients. Antihypertensives have been shown to cause decreased zinc levels through urinary loss. Our patient was taking both lisinopril and hydrochlorothiazide, which have been documented to cause a decrease in zinc levels. Because of his antihypertensive therapy, our patient will remain on zinc supplementation, even after complete clearance of his eruption. Commercial support: None identified.
Commercial support: None identified.
1287 A retrospective review of biopsy-proven calciphylaxis in a tertiary center in Singapore: Clinical features and prognosis in a Chinese population Yi Wei Yeo, MBBS, Singapore General Hospital, Singapore; Chay Mien Lim, Singapore General Hospital, Singapore; Hong Yi Koh, MBBS, Singapore General Hospital, Singapore; Thamotharampillai Thirumoorthy, MBBS, Singapore General Hospital, Singapore; Shiu Ming Pang, MBBS, Singapore General Hospital, Singapore; Haur Yueh Lee, MBBS, Singapore General Hospital, Singapore Background: Calciphylaxis is a small vessel obliterative vasculopathy characterized by mural calcification resulting in painful ischemic cutaneous ulceration. Mortality is high with a reported 1 year survival rate of 45.8% in Western populations. It is unclear if such high mortality is also present in other ethnic populations. We aimed to determine the epidemiologic profile, clinical features and outcomes of patients with calciphylaxis seen at an academic medical center in Singapore. Methods: A retrospective review of all biopsy-proven calciphylaxis cases seen by the Department of Dermatology, Singapore General Hospital from January 2012 to May 2014 was performed. Clinical and follow-up data were obtained from medical records. Results: A total of 16 biopsy-proven cases of calciphylaxis were identified. 14 were uremic while 2 were of nonuremic calciphylaxis. All patients were Chinese with a mean age of 57 years and a marked female preponderance of 93.8% (n ¼ 15). Of the uremic patients on dialysis, 8 (61.5%) were on hemodialysis while 4 (38.5%) were on peritoneal dialysis (mean duration ¼ 87.5 months). All patients had multiple comorbidities including diabetes mellitus, hypertension, ischemic heart disease and peripheral vascular disease. 2 patients with nonuremic calciphylaxis had systemic lupus erythematous and systemic sclerosis, respectively. In terms of clinical features, all patients presented with painful eschars and/or ulcers of the distal limbs with 2 (12.5%) also having proximal limb involvement. In addition, 8 (50%) had retiform purpura and 7 (44%) had induration suggestive of a panniculitis. 13 (81.3%) patients received intravenous sodium thiosulphate with 75% also being switched to noncalcium phosphate binders. 1 patient had hyperbaric oxygen therapy. 3 (18.8%) had surgical parathyroidectomy and 4 (25%) who were deemed medically unfit received cinacalcet instead. 4 (25%) had angioplasty and 5 (31.3%) had surgical debridement with 2 (12.5%) resulting in amputation. 13 patients were followed up beyond 1 year giving a 1- year mortality rate of 61.5% (n ¼ 8). All patients on peritoneal dialysis died by 13 months postdiagnosis.
530
Conclusion: This study shows that mortality remains high despite routine use of sodium thiosulphate and noncalcium phosphate binders. Our data also support previous studies that those on peritoneal dialysis may be associated with a poorer prognosis.
All trans-retinoic acid (ATRA)-induced Sweet syndrome in a leukemia patient Brandon Shutty, DO, Nova Southeastern University/Largo Medical Center, Largo, FL, United States; Richard Miller, DO, Nova Southeastern University/Largo Medical Center, Largo, FL, United States; David Dorton, DO, Nova Southeastern University/Largo Medical Center, Largo, FL, United States; George Gibbons, MD, Nova Southeastern University/Largo Medical Center, Largo, FL, United States; Jared Heaton, DO, Nova Southeastern University, Largo, FL, United States Sweet syndrome (SS), or acute febrile neutrophilic dermatosis, is a condition described as having painful erythematous papules and plaques along with the rapid onset of fever and leukocytosis. Generally this syndrome is idiopathic; however, associations exist with hematologic disorders, infection, connective tissue disease, or postexposure to drug therapy. All trans-retinoic acid (ATRA) is one such therapy rarely reported to be associated with the development of SS. This is exemplified by the case we present where a patient developed SS after treatment with ATRA for acute promyelocytic leukemia.
Commercial support: None identified.
Commercial support: None identified.
MAY 2015
J AM ACAD DERMATOL
AB143