Dermatologic Therapy, Vol. 28, 2015, 68–70 Printed in the United States · All rights reserved
© 2015 Wiley Periodicals, Inc.
DERMATOLOGIC THERAPY ISSN 1396-0296
THERAPEUTIC HOTLINE: LETTERS Necrobiotic xanthogranuloma with giant cell hepatitis, successfully treated with intravenous immunoglobulins Ana Filipa Pedrosa*†, Olga Ferreira*†, Ana Calistru*, Alberto Mota*†, Teresa Baudrier*, José Alexandre Sarmento‡, Herberto Bettencourt§ & Filomena Azevedo* Departments of *Dermatology and Venereology, ‡Gastroenterology and §Pathology, Centro Hospitalar São João EPE and †Faculty of Medicine, University of Porto, Porto, Portugal
A 44-year-old Caucasian woman with a year history of IgG kappa light-chain monoclonal gammopathy and giant cell hepatitis was referred to our department due to an arm lesion evolving for 2 months. On physical examination, an asymptomatic, erythematous-violaceous indurated plaque on the extensor surface of the left arm (Fig. 1A) and multiple yellowish, nontender, edematous, confluent papules on the periorbital area (Fig. 1B) were observed. The patient complained of recurrent red eye episodes and myalgia. Laboratory tests revealed elevation of transaminases and complement consumption; protein electrophoresis showed a diffuse increase in gamma globulins and a peak of free kappa light-chains; echocardiography was normal. Biopsies of the arm and periorbital lesions showed similar features, namely an infiltrate of histiocytes, including some multinucleated giant and foamy cells, extending into the hypodermis, foci of necrobiosis, cholesAddress correspondence and reprint requests to: Ana Filipa Bastos Pedrosa, MD, Department of Dermatology and Venereology, Centro Hospitalar São João EPE, Alameda Prof. Hernani Monteiro, Porto 4200-319, Portugal, or email: [email protected].
68
terol clefts, and a moderate lymphocytic infiltrate with occasional plasma cells (Fig. 1C–E). The diagnosis of necrobiotic xanthogranuloma (NXG) was established. Liver biopsy revealed hepatocytes with giant cell transformation, microvesicular steatosis, and intralobular necroinflammatory lesions. A bone marrow biopsy excluded a malignancy. Oral prednisolone (1 mg/kg/day) was started and later tapered off and replaced by oral budesonide (9 mg/day), yielding an initial reduction in transaminases values, but followed by another raising (ALT/AST 159/466, normal range
© 2015 Wiley Periodicals, Inc.
DERMATOLOGIC THERAPY ISSN 1396-0296
THERAPEUTIC HOTLINE: LETTERS Necrobiotic xanthogranuloma with giant cell hepatitis, successfully treated with intravenous immunoglobulins Ana Filipa Pedrosa*†, Olga Ferreira*†, Ana Calistru*, Alberto Mota*†, Teresa Baudrier*, José Alexandre Sarmento‡, Herberto Bettencourt§ & Filomena Azevedo* Departments of *Dermatology and Venereology, ‡Gastroenterology and §Pathology, Centro Hospitalar São João EPE and †Faculty of Medicine, University of Porto, Porto, Portugal
A 44-year-old Caucasian woman with a year history of IgG kappa light-chain monoclonal gammopathy and giant cell hepatitis was referred to our department due to an arm lesion evolving for 2 months. On physical examination, an asymptomatic, erythematous-violaceous indurated plaque on the extensor surface of the left arm (Fig. 1A) and multiple yellowish, nontender, edematous, confluent papules on the periorbital area (Fig. 1B) were observed. The patient complained of recurrent red eye episodes and myalgia. Laboratory tests revealed elevation of transaminases and complement consumption; protein electrophoresis showed a diffuse increase in gamma globulins and a peak of free kappa light-chains; echocardiography was normal. Biopsies of the arm and periorbital lesions showed similar features, namely an infiltrate of histiocytes, including some multinucleated giant and foamy cells, extending into the hypodermis, foci of necrobiosis, cholesAddress correspondence and reprint requests to: Ana Filipa Bastos Pedrosa, MD, Department of Dermatology and Venereology, Centro Hospitalar São João EPE, Alameda Prof. Hernani Monteiro, Porto 4200-319, Portugal, or email: [email protected].
68
terol clefts, and a moderate lymphocytic infiltrate with occasional plasma cells (Fig. 1C–E). The diagnosis of necrobiotic xanthogranuloma (NXG) was established. Liver biopsy revealed hepatocytes with giant cell transformation, microvesicular steatosis, and intralobular necroinflammatory lesions. A bone marrow biopsy excluded a malignancy. Oral prednisolone (1 mg/kg/day) was started and later tapered off and replaced by oral budesonide (9 mg/day), yielding an initial reduction in transaminases values, but followed by another raising (ALT/AST 159/466, normal range
