Malignant proliferating trichilemmal cyst of the scalp: histological aspects and nosology.

BACKGROUND: Malignant proliferating trichilemmal cyst is a rare tumour usually located on the scalp of elderly women. About 40 cases of malignant proliferating trichilemmal tumour have been documented. CASE REPORT: We report a case of a malignant proliferating trichilemmal cyst of the scalp in a 57-year-old woman. On the vertex she had a voluminous vegetated and multinodular tumour measuring 7 x 5 cm with spontaneous and abundant bleeding, and another lesion of the scalp corresponding to a trichilemmal cyst. Based on histopathological findings, the case was diagnosed as malignant proliferating trichilemmal cyst. CONCLUSION: Diagnostic, clinicoprognostic and histological features of this tumour are discussed. Treatment is not yet standardized given its rarity.

[Necrotic lesions of the face in Kawasaki disease]. / Lésions nécrotiques de la face au cours de la maladie de Kawasaki.

Kawasaki disease (KD) is a multisystemic vasculitis disorder in children. The dermatological manifestations are described as polymorphic and nonspecific. However, they are important for diagnosis. They correspond to 3 out of the 5 major criteria that may be associated with the disease's constant fever. We report the case of a child aged 3 years 8 months who showed necrotic lesions on the cheeks and the nose, associated with an erythematous, macular eruption on the rest of the body. Necrotic lesions have been exceptionally reported. These misleading cutaneous aspects should not delay or rule out the diagnosis of KD, especially in atypical or incomplete forms. Early diagnosis of KD should be made before the onset of coronary lesions.

[Primary cutaneous extraskeletal Ewing's sarcoma]. / Sarcome d'Ewing à localisation cutanée primitive.

BACKGROUND: Cutaneous extraskeletal Ewing's sarcoma is rare, being seen principally in children. We report a case of cutaneous sarcoma in the sole of the foot in a child. CASE REPORT: A 9-year-old child with no medical history of note was presenting a skin tumor for 3 months on the heel of the right foot. This tumor was burgeoning and painful and measured 3.5 cm in diameter; it was ulcerative at the surface and covered with a crust. Histological and immunohistochemical examinations confirmed the diagnosis of Ewing's sarcoma. Staging examinations proved negative and the patient underwent polychemotherapy, resulting in complete regression of the tumor. COMMENTS: Until 1998, 37 cases of cutaneous and subcutaneous Ewing's sarcoma were reported, being seen in 21 girls and 16 boys. Mean age at diagnosis was 15 years and mean tumor size was 3 cm (range: 1 to 12 cm). The tumors were observed throughout the body, being seen in the sole of the foot in 2 cases. Confirmation of the diagnosis was made by histological examination (malignant proliferation of small round cells in the dermis), immunohistochemical examination (CD99+) and cytogenetic analysis (translocation between chromosomes 22 and 11). The prognosis for cutaneous Ewing's sarcoma appears more favorable than that of Ewing's sarcoma in bone. Of the 37 patients treated, 7 had metastases and 2 presented relapse. Treatment for cutaneous Ewing's sarcoma, though not codified, consists of polychemotherapy associated with surgery and/or radiotherapy.

[Mycetomas in central Tunisia]. / Les mycétomes en Tunisie (région du centre).

INTRODUCTION: Mycetomas are inflammatory pseudo-tumors containing fungal or actinomycosic-type grains. They are frequent in tropical and subtropical countries and unknown in Tunisia. PATIENTS AND METHODS: We conducted a retrospective study of 12 cases of mycetoma registered in the Dermatological department of the university hospital in Sousse (central Tunisia) over a period of 27 years, from 1974 to 2001. The diagnosis was confirmed by anatomopathological and/or mycological examination. RESULTS: The mean age at the onset was of 49 years and the sex ratio of 1. A notion of a traumatism was reported in two cases and eight patients had various agricultural activities. The mean duration of progression was of eight years. The localization was the foot in 10 cases. The mycetoma was of actinomycosic origin in 10 cases, due to Actinomadura madurae in nine cases, to Nocardia spp in one case and of fungal origin in 2 cases:Pseudoallescheria boydii in one case and Madurella mycetomi in the other. Antibiotic therapy was associated with surgical exeresis in nine cases and amputation in the other two cases. COMMENTS: Confrontation of our results with those of Tunisian series and a review of the literature, helped to specify the clinico-epidemiological characteristics and progression of mycetoma in Tunisia. These characteristics are: the rareness of the infection, the relative frequency of affection in women, the proximal involvement of the foot, the frequency of agricultural activity and the rareness of traumatic past history, the predominance of the actinomycosic origin due to Actinomadura madurae, and the need to associate surgical exeresis with the medical treatment or amputation in order to stop the progress of the disease.

[Juvenile colloid milium]. / Milium colloïde juvénile.

INTRODUCTION: Colloid Milium is a rare disease. It is characterized by the development, on sun-exposed areas, of clusters of yellow and translucid, hard papules, that seeps a gelatinous substance when opened. This dermatitis predominantly affects elderly patients and, exceptionally, children. We report a case of juvenile colloid milium. OBSERVATION: A 14 year-old boy consulted for papular, translucid or amber-brown lesions, occasionally hemorrhagic on the cheeks, nose upper lip and the upper edge of the helix of both ears. He was born to consanguineous parents. Living in a rural area, he was exposed to sun during agricultural activities. The lesions had progressed since he was 6 years old and were exacerbated in the summertime. There was no similar case in the family. Histological exploration of a papular lesion showed areas of atrophic epidermis and the presence of a few vacuolized keratinocytes. The epidermis was lifted by nodular lumps of amorphous eosinophilic material deposited in the superficial dermis. Staining with Congo red was negative. DISCUSSION: Juvenile colloid milium is a benign but unesthetic dermatitis. The inducing role of sun exposure is obvious, as in colloid milium of adults, but other pathogenic hypotheses also exist.

[Skin aging]. / Le vieillissement cutané.

The skin aging is the result of the association of seven factors; chronological factor, genetic factors, exposure to ultraviolet rays (photodamage), behaviour factors, endocrinous, catabolics and mechanics factors. The treatment combine sun protection measures, topical treatment, dermatological and surgical techniques (peelings, resurfacing laser, injections of collagen, botulic toxin...). The authors intend to study clinical and anatomy-physiology aspect of skin aging, as well as different treatments.

[Giant cell fibroblastoma associated with Darier-Ferrand dermatofibrosarcoma in an adult]. / Association d'un fibroblastome à cellules géantes et d'un dermatofibrosarcome de Darier-Ferrand chez un adulte.

INTRODUCTION: Giant cell fibroblastoma is a rare mesenchyma tumor of childhood having many similarities with dermatofibrosarcoma protuberans in adults. OBSERVATION: We report the case of a 28-year-old woman presenting a subcutaneous inter-mammary mass associating both tumors. Immunohistochemistry showed an expression of CD 34 only by dermatofibrosarcoma protuberans cells. DISCUSSION: It is important to stress: the rarity of this association, the difficulty to confirm the diagnosis and to establish the links between these two tumors: simple association (as in our case), transformation or recurrence of giant cell fibroblastoma in dermatofibrosarcoma protuberans.

[Kaposi's sarcoma and hypereosinophilic syndrome]. / Sarcome de Kaposi et syndrome hyperéosinophilique.

The authors report the case of a 73-year-old patient with severe Kaposi's sarcoma associated with an idiopathic hypereosinophilic syndrome. This association has not been reported until today and incites us to report this case.