J.-L. Verret

Hidradénome nodulaire malin et hidradénomes nodulaires multiples chez un malade hypogonadique

Resume Introduction Nous rapportons une observation d’hidradenomes nodulaires benins multiples associes a un hidradenome nodulaire malin chez un malade hypogonadique. Observation Chez un homme de 49 ans etaient survenus 11 hidradenomes nodulaires benins de la region pectorale. Un hidradenome nodulaire malin etait recemment apparu dans cette region, a distance des hidradenomes nodulaires benins preexistants. Il existait chez ce malade un hypogonadisme peripherique en rapport avec une cryptorchidie congenitale bilaterale a l’origine d’une gynecomastie par « hyperœstrogenie relative ». Des recepteurs aux œstrogenes etaient mis en evidence en grand nombre sur chacun des hidradenomes nodulaires benins. Le bloc de l’hidradenome nodulaire malin etait egare, ne permettant pas la recherche de ces recepteurs. Discussion Les observations d’hidradenomes nodulaires multiples sont tres rares : nous n’en avons recense que deux. L’association d’un hidradenome nodulaire malin a de multiples hidradenomes nodulaires benins n’a, a notre connaissance, jamais ete rapportee. Neanmoins, histologiquement, des formes de transition existent. L’existence d’anomalies endocriniennes associees a ce type de tumeurs n’a jamais ete rapportee. La presence de recepteurs aux œstrogenes a en revanche parfois ete mise en evidence dans des hidradenomes nodulaires benins. La pathologie endocrinienne chez ce malade a peut-etre joue un role dans la multiplicite de ses hidradenomes, mais ceci ne peut etre prouve.

Hereditary leukonychia totalis, acanthosis-nigricans-like lesions and hair dysplasia: a new syndrome?

Leukonychia is an ungueal discoloration or dyschromia. The hereditary form is rare. In the observations reported in the literature, leukonychia was total or sub-total, and was sometimes associated to other various symptoms. We report an original observation of hereditary leukonychia totalis in a father and two of his children, associated with acanthosis-nigricans-like lesions and hair dysplasia. These symptoms were also present in eight other members of the same family.

Lichen sclereux acral

BACKGROUND Lichen sclerosus et atrophicus rarely affects the feet or hands and in this case, it is generally part of widespread cutaneous involvement. We report a case of lichen sclerosus et atrophicus involving only the extremities and the vulvar and perigenital area. PATIENTS AND METHODS A 56-year-old woman presented with lesions of the hands and feet, with ivory white papules on the dorsal aspect of the feet and the distal phalanx of the fingers, a few small keratotic papules with central depressions in the hollow of the palms, erythema on soles and thenar and hypothenar eminences. Further examination revealed lichen sclerosus et atrophicus of the vulva and genitocrural skinfolds. Histological study of these various cutaneous lesions yielded similar results and revealed the typical features of lichen sclerosus et atrophicus. DISCUSSION A few cases of lichen sclerosus et atrophicus confined to the hands and/or feet have been reported, involving the palms and soles or nail folds, but none has so far affected the genitalia. To our knowledge, no cases of lichen sclerosus et atrophicus involving both faces of the hands and feet and the genital region have ever been reported.

Présentation inhabituelle d’une myofibromatose cutanée infantile en plaque unique ulcérée

Resume Introduction La myofibromatose infantile est une tumeur rare d’aspect fibrovasculaire, de localisation solitaire ou multicentrique, cutanee ou plus souvent sous cutanee, parfois osseuse, voire viscerale, survenant generalement avant l’âge de 2 ans. Nous rapportons une observation de myofibromatose infantile du nourrisson dans une forme clinique atypique en plaque unique a centre ulcere ayant succede a des papulo-nodules groupes. Observation Un nourrisson etait amene en consultation pour l’apparition de papulo-nodules asymptomatiques localises du dos. L’examen histologique apres biopsie partielle montrait un aspect d’histiocytofibrome faisant porter le diagnostic d’histiocytofibromes multiples groupes du dos. Les papulo-nodules confluaient un an plus tard pour former une large plaque au centre ulcere. L’extension progressive et l’absence de cicatrisation conduisaient a une exerese chirurgicale permettant de poser histologiquement le diagnostic de myofibromatose. Discussion Le diagnostic de myofibromatose infantile est difficile tant cliniquement qu’histologiquement et repose sur une bonne confrontation anatomoclinique. Les aspects cliniques peuvent etre tres divers. A notre connaissance, la myofibromatose a type de plaque ulceree unique n’a jamais ete rapportee dans la litterature.

Angiosarcome cutané de jambe sans lymphœdème associé

BACKGROUND Cutaneous angiosarcoma is a rare aggressive vascular neoplasm with a poor prognosis, seen chiefly in elderly subjects and usually on the scalp or face. The present case is original because of its localization on the leg without any chronic lymphoedema and because of the long survival period. The treatment modalities are discussed. CASE REPORT An 87-year-old woman presented with a rapidly growing large deep-purple ulcerated tumour on the anterior aspect of the leg. In addition, two nodules with a similar aspect appeared on the outer surface of the foot. Histological examination showed vascular channels lined with atypical cells consistent with a diagnosis of angiosarcoma. Computed tomography revealed no metastases. Amputation was performed at the thigh and there was no recurrence 30 months later. DISCUSSION The leg is a rare site of cutaneous angiosarcoma. Treatment usually consists of surgical excision with wide margins followed by radiotherapy, but in some cases amputation is unavoidable.