Résumé
Interstitial granulomatous dermatitis (IGD), a rare dermatological disorder was first described by Ackerman et al, in the year 1993. It is characterized by a heterogeneous clinical spectrum and a specific histopathological pattern. It has been described in association with a number of auto-immune disorders, drugs, fungal infections, and malignancies. It may manifest as papules, patches, plaques, nodules, annular lesions, or less frequently, as described classically, as indurated linear subcutaneous cords. This case is being reported as interstitial granulomatous dermatitis is rare dermatological manifestation of rheumatoid arthritis and presentation as large ulcerated skin lesions is further extremely rare. In this case report we describe a 67-year-old gentleman with Seropositive Rheumatoid arthritis. He presented to our hospital with complaints of multiple well defined skin lesions with pus discharge over lower limbs, trunk and upper limbs. Biopsies from left upper limb and abdomen showed focal necrobiosis with surrounding granulomatous inflammation with rare perivascular granulomas. He was subsequently diagnosed to have interstitial granulomatous dermatitis based on histopathological examination. Interstitial granulomatous dermatitis has been described to be associated with a number of autoimmune conditions and drugs. It is very important for clinicians to have knowledge about this rare skin condition as it may be heralding presentation of a serious underlying condition like lymphoproliferative disorders or solid organ malignancies. There is no specific treatment and causative disease has to be diagnosed and targeted.
Résumé
Palisaded neutrophilic and granulomatous dermatitis (PNGD) is a newly defined entity that includes various clinical entities. Histopathologically, this disease is characterized by a granulomatous inflammation with or without leukocytoclastic vasculitis. PNGD shows vasculitic lesions in the early stage, with palisaded granulomatous lesions and dermal fibrosis with minimal leukocytoclastic debris appearing in the late stage. It is frequently associated with rheumatoid arthritis, lupus erythematosus, and other autoimmune diseases. A 14-year old Korean girl presented with multiple erythematous nodules and plaques on both elbows and knees that were present for 2 years prior to the initial visit. Clinically, she had multiple arthritis with morning stiffness and decreased C3 levels, as well as positive results for antinuclear antibodies, the lupus anticoagulant test, and anti-beta 2 glycoprotein I (IgG). Skin biopsy findings from the skin lesion indicated that the foci of degenerated collagens were palisaded with histiocytes throughout the dermis and neutrophils with leukocytoclasia infiltrated with fibrin, with mucin at the center of the lesion. Based on the SLICC 2012 criteria, we diagnosed her condition as PNGD in systemic lupus erythematosus. She was treated with hydroxychloroquine and topical application of 0.1% tacrolimus ointment, which resulted in the remarkable flattening of the skin lesions after 6 months of follow-up. Herein, we report a case of PNGD that may have been an indicator of systemic lupus erythematosus.
Sujets)
Femelle , Humains , Anticorps antinucléaires , Arthrite , Polyarthrite rhumatoïde , Maladies auto-immunes , Biopsie , Collagène , Dermatite , Derme , Coude , Fibrine , Fibrose , Études de suivi , Glycoprotéines , Histiocytes , Hydroxychloroquine , Inflammation , Genou , Inhibiteur lupique de la coagulation , Lupus érythémateux disséminé , Mucines , Granulocytes neutrophiles , Peau , Tacrolimus , VasculariteRésumé
Palisaded neutrophilic and granulomatous dermatitis (PNGD) is an uncommon condition that has been reported under several different names in association with various disorders such as rheumatoid arthritis and systemic lupus erythematosus. We report a 60-year-old man with rheumatoid arthritis who presented with two, erythematous, asymptomatic nodules on the buttock. The histologic examination showed that degenerated collagen, neutrophilic dust, neutrophils, fibrin, and mucin were present in the centers of the palisades of histiocytes throughout the dermis. After initiation of intralesional steroid injections, the lesions improved. The lesions recurred 2 months later, but, as before, improved after treatment with intralesional steroid injections.
Sujets)
Humains , Adulte d'âge moyen , Polyarthrite rhumatoïde , Fesses , Collagène , Dermatite , Derme , Poussière , Fibrine , Histiocytes , Lupus érythémateux disséminé , Mucines , Granulocytes neutrophilesRésumé
Palisaded neutrophilic and granulomatous dermatitis (PNGD) is an uncommon skin eruption associated with immune-mediated connective tissue diseases such as rheumatoid arthritis, lupus erythematosus, and Behcet's disease. This condition is also described as Winkelmann's granuloma, linear subcutaneous bands, rheumatoid papules, rheumatoid neutrophilic dermatitis, interstitial granulomatous dermatitis with cutaneous cords and arthritis, or Churg-Strauss disease. PNGD shows variable clinical manifestations and is characterized histopathologically by step pattern of early leukocytoclastic vasculititis, progressing to form palisaded granulomatous inflammation, and surrounding zones of necrobiosis in the later stages. We report a 66-year-old woman with multiple skin nodules, who had concomitant rheumatoid arthritis. Histopathologic findings revealed palisaded neutrophilic and granulomatous dermatitis.