DP18 A rare association between interstitial granulomatous dermatitis and myelofibrosis

Published Date: 23rd December 2019

Publication Authors: Low SE, Ngan K

Abstract

A 61-year-old woman presented with an 18-month history of a widespread eruption, which had started on her face and later spread to involve her neck, chest, upper back and upper limbs. She had been previously been treated for rosacea with metronidazole gel (MetroGel) and 1% hydrocortisone cream without improvement. Her medical history included eczema/ psoriasis limited to the hands, valve replacement surgery, hypertension and gastro-oesophageal reflux, and medications included ramipril, warfarin and lansoprazole. Examination of the affected areas revealed multiple annular and discoid-looking infiltrative, erythematous plaque. The initial clinical appearance was highly suspicious for Jessner lymphocytic infiltrate or discoid lupus. Blood investigations revealed mild anaemia with significant lymphopenia and autoimmune profile was largely unremarkable including normal complements and negative antinuclear antibody, double-stranded DNA and extractable nuclear antigen. Skin biopsies taken from the left upper arm and left forehead revealed palisaded interstitial histiocytes and focal leucocytoclastic vasculitis, in keeping with a diagnosis of interstitial granulomatous dermatitis (IGD). This prompted a further review of the patient's history because of the possible association of this condition with an underlying malignancy. The patient reported a 3-stone weight loss and night sweats over the preceding 2 years. These symptoms raised the possibility of an underlying haematological disorder, prompting a whole-body computed tomography scan to be arranged, which revealed hepatosplenomegaly and enlarged mesenteric lymph nodes. The patient was urgently referred to the local Haematology team and subsequently underwent a bone-marrow trephine biopsy, molecular genetics and cytogenetic analysis. She was diagnosed with myelofibrosis, which was further supported by the identification of a JAK2 mutation and a deletion mutation on the long arm of chromosome 20. In the meantime, she was also commenced on hydroxychloroquine, which resulted in marked improvement of her skin rash. IGD is a rare inflammatory dermatosis characterized by the histopathological findings of a predominantly histiocytic infiltrate interposed between collagen bundles in the reticular dermis (Garcia-Rabasco A, Esteve-Martinez A, Zaragoza-Ninet V et al. Interstitial granulomatous dermatitis in a patient with lupus erythematosus. Am J Dermatopathol 2011; 33: 871-2). Clinically, it presents as a polymorphic eruption typically with erythematous papules and plaques that may be discoid, annular or cord-like. It can be associated with connective tissue disease or underlying malignancy. There have been sparse case reports in literature linking IGD with myeloproliferative disorders. To our knowledge, our case represents the first reported case in the U.K. of IGD associated with myelofibrosis.

Qazi, E; Phillips, D; Low, SE; Khirwadkar, N; Ngan, K. (2019). DP18 A rare association between interstitial granulomatous dermatitis and myelofibrosis: An interesting case report . British Journal of Dermatology. 181 (S1), 125

« Back