[Autoimmune progesterone dermatitis]. / Dermatose auto-immune à la progestérone.

BACKGROUND: Autoimmune progesterone dermatitis (AIPD) is caused by an immune reaction to endogenous progesterone following exposure to exogenous progesterone, especially in oral contraceptives and intrauterine devices. Skin eruption develops cyclically during the luteal phase of the menstrual cycle when progesterone levels are elevated. The resultant clinical symptoms are frequently confused with other forms of dermatosis. We describe a young woman presenting AIPD. PATIENTS AND METHODS: A 23-year-old woman had a history of a chronic pruritic papular-vesicular eruption on the trunk, present since the onset of puberty and unsuccessfully treated with antihistamines and topical steroids. Skin lesions normally began a few days before menstruation and resolved a few days afterwards. Intradermal testing using progesterone was positive, thereby confirming the diagnosis. The patient's skin lesions improved under oral contraceptives designed to inhibit ovulation and there was no relapse during subsequent menstrual cycles. DISCUSSION: AIPD is a rare autoimmune disorder. The diagnostic criteria of AIPD include recurrent cyclical worsening of skin lesions and symptomatic improvement after inhibition of progesterone secretion by suppression of ovulation. The pathogenesis is unclear. Positive response to various tests with progesterone suggests involvement of various mechanisms such as antibodies and/or cell-mediated immune reactions. CONCLUSION: AIPD is a rare disease that can seriously affect patients' quality of life and which may even be life-threatening in certain cases.

[Childhood cutaneous Abrikossoff tumor]. / Tumeur d'Abrikossoff purement cutanée chez 2 enfants.

INTRODUCTION: Granular cell tumor (GCT), also known as Abrikossoff tumor, is a rare and benign neoplasm that more commonly affects the oral cavity in adults. We report here 2 childhood cases of a cutaneous granular cell tumor. CASE REPORTS: An 8- and a 12-year-old girl, with no past medical history, presented with pigmented nodular lesions located, respectively, in the left scapular region and the left iliac crest. Histological examination showed sheets and clusters of infiltrating tumor cells with morphologic and immunohistochemical features consistent with granular cell tumor. In both cases, the lesions were excised with 2-cm margins. The patients are presently being reviewed at 6-month and 2-month intervals to evaluate for recurrence and any malignant transformation. They were in good health with no signs of further tumor development. DISCUSSION: GCT is a relatively uncommon benign neoplasm probably of neural origin derived from Schwann cells. The tongue and buccal mucosa are commonly affected. It develops between the second and sixth decades of life, more frequently among women and blacks. It can also occur in childhood, but this is rare. The cutaneous forms, as in the present observations, are exceptional. Benign granular cell tumours are generally seen as a solitary asymptomatic nodule less than 3 cm in size involving the subcutaneous or submucosal tissues. Histologically, the tumor is not encapsulated but is well circumscribed. The tumor cells may show a degree of infiltration of the surrounding connective tissue. The cells are polygonal, have abundant cytoplasm, are granular and weakly eosinophilic. The nuclei are small, vesicular, and highly chromatic. Mitotic figures are rare. A key element for the histological diagnosis is the expression of S100 protein, neuron specific enolase (NSE), and vimentin on immunohistochemistry. The treatment of choice is a conservative surgical excision of the lesion. However, as the GCT has a poorly defined margin, it is suggested that the tumor should be excised along with portions of adjacent tissue. A low rate of recurrence of the lesion has been reported. CONCLUSION: The specific value of these rare cases is the occurrence in 2 children and the cutaneous location.