Papular mucinosis associated with AIDS: Response to isotretinoin☆,☆☆,★

La mucinose papuleuse est une affection dermatologique rare, caractérisée par des papules, nodules ou plaques dues à des dépôts de mucine dans le derme, et à un certain degré de fibrose, sans dysfonctionnement thyroïdien.

Un homme de 42 ans consultait pour des lésions papuleuses symétriques du visage, de la nuque et des épaules. Ces lésions s’étaient étendues progressivement depuis environ 20 ans. Les examens biologiques étaient normaux. L’examen histologique montrait des dépôts diffus de mucine dans le derme superficiel et moyen, des faisceaux de collagène épais, une prolifération de fibroblastes et un discret infiltrat mononucléé périvasculaire. L’ensemble de ces éléments faisait retenir le diagnostic de mucinose papuleuse.

La particularité de cette observation est l’atteinte prédominante du visage et l’étendue des lésions. Selon la classification réactualisée par Rongioletti, ce malade avait une forme atypique, originale, de mucinose papuleuse sous la forme « discrète papuleuse » avec atteinte du visage. Le recul de 20 ans sans atteinte systémique ou paraprotéine, nous fait penser que cette forme localisée n’évoluerait pas vers la forme généralisée ou scléromyxœdème.

Papular mucinosis is a rare dermatological disorder characterized by papules, nodules or plaques resulting from mucin deposits in the dermis, and to a certain degree, from fibrosis, without thyroid dysfunction.

A 42-year-old man consulted for symmetrical papular lesions on the face, neck and shoulders. The lesions had gradually spread over some 20 years. Laboratory findings were normal. Histopathologic examination showed diffuse mucin deposits in the superficial and middle layers of the dermis, thick bands of collagen, proliferation of fibroblasts and mild perivascular infiltration by mononuclear cells. All of these findings pointed to a diagnosis of papular mucinosis.

The specific interest of this case is the predominant involvement of the face and the spread of the lesions. According to the updated Rongioletti classification, this patient is presenting an atypical and novel form of papular mucinosis of a “mild papular” type with facial involvement. The 20-year history without systemic involvement or paraprotein suggests to us that this localized form will not develop into the systemic form or scleromyxoedema.

Isotretinoin (13-cis-retinoic acid) is a retinoid that has been used over the past 2 decades to treat a wide variety of dermatologic conditions, some with great success. Although it is beneficial in many skin conditions, the side effects and toxicities of oral retinoids require careful monitoring by experienced physicians. The clinical applications of oral retinoids continue to expand both within and beyond the field of dermatology. (J Am Acad Dermatol 2001;45:S150-7.)

Scleromyxedema is a variant of papular mucinosis characterized by fibroblast proliferation and mucin deposition in the dermis. Historically, it has been very difficult to treat and can cause significant morbidity and mortality with systemic involvement. We describe a case of a woman with scleromyxedema and systemic manifestations treated with interferon alfa. Her skin responded very well to therapy within 3 months; however, her systemic manifestations showed little change. We conclude that interferon alfa may be a useful therapy for patients with scleromyxedema, particularly if the disease process is limited to the skin. (J Am Acad Dermatol 1999;40:303-7.)