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Lupus
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Review : A peculiar form of amicrobial pustulosis of the folds associated with systemic lupus erythematosus and other auto-immune diseases

S. Lagrange

Services de Médecine Interne, Groupe Hospitalier Pitié-Salpêtrière, 47-83 Boulevard de l'Hôpital 75651, Paris cedex 13, France

O. Chosidow

Services de Médecine Interne, Groupe Hospitalier Pitié-Salpêtrière, 47-83 Boulevard de l'Hôpital 75651, Paris cedex 13, France

JC Piette

Services de Médecine Interne, Groupe Hospitalier Pitié-Salpêtrière, 47-83 Boulevard de l'Hôpital 75651, Paris cedex 13, France

B. Wechsler

Services de Médecine Interne, Groupe Hospitalier Pitié-Salpêtrière, 47-83 Boulevard de l'Hôpital 75651, Paris cedex 13, France

P. Godeau

Services de Médecine Interne, Groupe Hospitalier Pitié-Salpêtrière, 47-83 Boulevard de l'Hôpital 75651, Paris cedex 13, France

C. Frances

Services de Médecine Interne, Groupe Hospitalier Pitié-Salpêtrière, 47-83 Boulevard de l'Hôpital 75651, Paris cedex 13, France

Amicrobial pustulosis of the folds is a new entity which was initially described in association with systemic lupus erythematosus (SLE). We report four cases of this rare pustulosis occurring in the course of various auto-immune disorders.

Dermatological features were similar in all patients. The primary lesions were small pustules which coalesced and formed erosive areas underlined with pustular lesions. They were localized in the folds. Three of the four patients had also isolated pustules on the scalp. Spongiform pustules were histologically present. A primary bacterial or fungal infection was ruled out by cultures on appropriate media. Serum calcium level was normal. Associated auto-immune diseases were: systemic lupus erythematosus, incomplete systemic lupus erythematosus, 'idiopathic' thrombocy topenic purpura and myasthenia gravis without thymoma (one of each). The relationship between this peculiar form of pustulosis and autoimmunity remains to be elucidated. The pustulosis did not respond to antibiotics but disappeared with systemic or topical corticosteroids.

Key Words: amicrobial pustulosis • folds • systemic lupus erythematosus • auto-immune diseases

Lupus, Vol. 6, No. 6, 514-520 (1997)
DOI: 10.1177/096120339700600607
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