Dermatitis herpetiformis and rheumatoid arthritis

Archana Singal, Sambit Nath Bhattacharya, Manik Chandra Baruah

 Department of Dermatology and STD, University College of Medical Sciences and GTB Hspital, Shahdara, Delhi- 110 095, India

Correspondence Address:
Archana Singal
8 16, F -1, Dilshad Garden Delhi - 110 095
India

Introduction

Dermatitis herpetiformis (DH) is an uncommon disease characterized by the presence of intensely pruritic, chronic, recurrent, grouped papulovesicular lesions on erythematous and/or urticarial background. Though gluten sensitive enteropathy which may be asymptomatic, is a consistent association, many other endocrinological, connective tissue, liver, lung, skin diseases like lichen planopilaris, alopecia areata, vitiligo have been found to be associated with DH.[1],[2]

Case Report

A 35 - year -old woman, deaf and dumb since birh, was seen for the complaints of intensely itchy skin lesions on back, buttock and limbs, of seven months duration. There was history of joint pain in hands and feet for seven years, which worsened every winter. Patient had developed progressive deformity of hands and feet despite being intermittently on systemic corticosteroid therapy [Figure - 1] and [Figure - 2]. On examination she had excoriated and crusted erosions with very few papulovesicular lesions distributed extensively on back [Figure - 3], buttock, forearm and legs. A few lesions had healed with scarring an hypopigmentation. Face, scalp, flexural areas, palms, soles and mucosae were spared.

On investigation ESR was 80mm in first hour and rheumatoid factor was positive. X-ray hands and feet revealed changes of rheumatoid arthritis. Other haematological investigations including haemogram, serum biochemistry and urinalysis were within normal limits. Histopathology of an intact vesicular lesion from upper back revealed subepidermal bulla with neutrophilic abscesses at the tip of dermal papillae. A diagnosis of DH was made and the patient was put on tablet dapsone 100 mg twice daily and advised gluten free diet. There was a dramatic reduction in the severity of itching and arrest of new lesions within 72 hours. For symptomatic relief of joint pains indomethacin 25 mg 8 hourly after food was administered.

Discussion

Association of DH with various other autoimmune diseases and immune system mediated skin diseases have been described in various reports. In a 10 year cohort study by Reunala and Colin,[1] of 305 patients with DH, 9.5% of patients developed various autoimmune diseases and of this 0.7% developed rheumatoid arthritis. Other reports describe as- sociation of DH with systemic lupus erythematosus by Moncada[3] and Davies,[4] thyroid disease, dermatomyositis and Sjogren′s syndrome by White and Tesar[5] and rheumatoid arthritis by Rothwell.[6] [Table - 1].

The association of other autoimmune diseases and presence of different organ specific and non organ specific antibodies reported in DH patients by various workers[7],[8] further supports the hypothesis of a similar or overlapping dysregulation in the control of the complex immune regulatory mechanisms, in the pathogenesis of these diseases.

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