[Transitory apocrine hidrocystomatosis of the scrotum]. / Hidrocystomatose apocrine transitoire du scrotum.

BACKGROUND: Apocrine hidrocystomas are benign cystic tumors that develop from apocrine gland proliferation. In most cases, they are translucent solitary lesions of the face, generally found in the periorbital region, on the scalp or on the neck. More rarely, apocrine hidrocystomas may be multiple and appear on the ears, trunk, shoulders and genital area. They generally appear in adulthood, with only a few pediatric cases being reported, of which three in the genital area, with a solitary case of multiple hidrocystomas of the scrotum, although no cases of spontaneous involution of hidrocystomas have previously been reported. PATIENTS AND METHODS: Two boys aged 4 and 6 months were seen in consultation for small sub-millimeter size, subcutaneous, black lesions on the scrotum that appeared in the weeks following birth. Histological examination of these lesions resulted in a diagnosis of apocrine hidrocystoma. The children were seen again a few weeks later and the skin lesions had totally disappeared. We report two cases of multiple apocrine hidrocystomas on the scrotum with spontaneous involution diagnosed in a 4- and a 6-month-old boy. DISCUSSION: Apocrine hidrocystomas are rare benign adnexal tumors that develop from apocrine sweat glands. They are considered as cystic proliferations of the apocrine glands rather than simple retention cysts. The main differential diagnosis of the rare cases of multiple apocrine hidrocystomas are eccrine hidrocystomas. The treatment of such lesions is based on surgical excision if they are isolated, daily application of topical atropine 1%, or CO2 laser for multiple apocrine hidrocystomas.

[Orf nodules and immunosuppression: a case report and review of therapeutics]. / Nodules d'Orf et immunosuppression : rapport d'un cas et revue des therapeutiques.

In immunocompromised patient, parapoxvirus infection can be extensively necrotic and recurrent evolution. We describe a case of Orf nodule in a liver transplanted woman. We will consider the therapeutic options in case of infections by parapox in immunosuppressive patients, as described in the medical literature. In our specific case, local application of cidofovir (concentration of 1 %) together with local antiseptic solution, povidone iodine, led to complete remission of the lesion without any sign of toxicity. Finally, we will consider the therapeutic use of local cidofovir.

[Palmoplantar neutrophilic eccrine hidradenitis with general extension in a child in remission after acute lymphoblastic leukemia]. / Hidradénite eccrine neutrophilique palmo-plantaire avec extension généralisée chez un enfant en rémission d'une leucémie aiguë lymphoblastique.

BACKGROUND: Neutrophilic eccrine hidradenitis (NEH) is a form of neutrophilic dermatitis characterized by tender erythematous and painful papules involving the trunk, extremities and face. The generalized form is associated with malignant hemopathies. The palmoplantar form occurs in children without any context of malignancy. Histology shows a neutrophilic infiltrate surrounding and infiltrating the eccrine glands associated with vacuolar degeneration and necrosis of the epithelial secretory portion. PATIENTS AND METHODS: We report the case of a 4-year-old girl with palmoplantar HEN progressing to the generalized form while in remission from acute lymphoblastic leukemia. DISCUSSION: Progression of HEN from the palmoplantar form to the generalized form has never been published previously in the literature.

Oral ulceration: an unusual manifestation of Churg-Strauss syndrome.

Churg-Strauss Syndrome (CSS) is a relatively rare entity characterized by asthma, transient pulmonary infiltrates, eosinophilia and systemic vasculitis. Oral ulceration is a possible clinical manifestation of some systemic vasculitides, such as Wegener's granulomatosis (WG) or giant cell arteritis, but has never been reported with Churg-Strauss syndrome. We report the first observation of a palatine ulceration in a 15-year-old girl with Churg-Strauss syndrome.

[Acquired lymphangiectasias or mammary dermolytic blisters with spontaneous resolution]. / Lymphangiectasies acquises ou bulles dermolytiques mammaires spontanément résolutives.

INTRODUCTION: Spontaneous occurrence of hemorrhagic blisters on the lower side of the breasts is an unusual clinical presentation of acquired lymphangiectasia. CASE REPORT: A 64 Year-old and a 85 Year-old woman had developed recurrent bleeding blisters in the sub-mammary region for several Months. Histological, immunohistochemical and electron microscopic examination revealed a subpapillary dermal bullous dehiscence, parallel to the epidermis, which was connected to lymphangiectasias of the superficial dermis. Their extreme dilatation and rupture were probably responsible for the clinical inflammatory and bleeding aspect of the lesions, which have not recurred after 1 and 3 Years, respectively. DISCUSSION: The presence of subpapillary inflammatory lymphangiectasias might be responsible for recurrent dermal blister formation. The reason for the presence of these hemorrhagic lymphangiectasias, restricted to the sub-mammary location and their spontaneous regression after several flare-ups, remain unclear. The unusual clinical presentation of the lesions observed in these two women constitutes a differential diagnosis of acquired dermolytic blisters.

Multiple HPV-positive basal cell carcinomas on the abdomen in a young pregnant woman.

Basal cell carcinoma (BCC) is the most common malignant skin tumour. In the last few years, the incidence of multiple BCC has also increased in young patients. We describe the clinical case of a young 29-year-old woman who developed 7 BCC on her abdomen during her first pregnancy and 4 other similar tumours 2 years later during her second pregnancy, all located on the abdomen. Polymerase chain reaction revealed the presence of numerous human papillomavirus DNA sequences. To our knowledge, such a clinical presentation has not been previously reported. Different physiopathological considerations are discussed.

Multinucleate cell angiohistiocytoma: report of two cases with no evidence of human herpesvirus-8 infection.

Multinucleate cell angiohistiocytoma (MCA) is a vascular tumor of unknown pathogenesis. Possible misinterpretation of this disorder with Kaposi's sarcoma (KS), a human herpesvirus-8 (HHV-8)-associated tumor, prompted us to look for this virus in two women with MCA. None of the multiple skin specimens obtained from both our patients produced amplified HHV-8 DNA. Using a cell culture methodology similar to that used for KS, we established cell cultures from MCA lesions. While KS spindle cells are known to exhibit in vitro invasive properties and can be grown up to more than 20 passages, the MCA-derived cells were short-lived and were not able to traverse basement membranes. Taken together, our data support the hypothesis that MCA is not a neoplasm but a benign vascular proliferation which is clearly distinguishable from KS.

A sporadic case of progressive mucinous histiocytosis.

Hereditary progressive mucinous histiocytosis is a rare autosomal dominant non-Langerhans cell histiocytosis. We describe a sporadic case of this syndrome in a 64-year-old woman who had multiple dark-red dome-shaped papulonodules located mainly on the back of her hands, forearms and thighs. Light microscopy revealed a circumscribed upper dermal aggregate of ovoid or spindle-shaped histiocytes with abundant mucin deposition. Iron deposits and numerous mast cells were scattered throughout the tumour but giant cells were rare. Electron microscopy revealed a high number of zebra bodies and myeloid bodies in the cytoplasm of the histiocytes. Immunohistochemistry showed positive labelling with alpha-1 antitrypsin, Factor XIIIa and CD68, while CD1a, CD34 and S100 protein were negative. The differential diagnosis of histiocytic syndromes is discussed.

An unusual case of squamous cell carcinoma of the nose.

A 49-year-old man presented with a tumoral lesion of the tip of the nose which had been manifest for 2 months. Within a few weeks, the tumour increased in size and became infiltrated. The biopsy showed a squamous cell carcinoma. Treatment consisted of a radical surgical excision.

Hermansky-Pudlak syndrome: a case report and discussion.

Hermansky-Pudlak syndrome is a rare, inherited, autosomal recessive disease. Diagnosis is based on a triad of signs: oculocutaneous albinism, a hemorrhagic tendency due to a platelet disorder, and an accumulation of lipopigments in different organs, particularly the medullary macrophages. We describe a child with the characteristic findings of this syndrome, which often goes unrecognized because of the discrete nature of the cutaneous and hemorrhagic manifestations. This diagnosis is important because of the risk not only of hemorrhage but also of granulomatous colitis and long-term pulmonary fibrosis.

Baboon syndrome.

An 18-month-old patient developed Baboon syndrome after oral treatment with erythromycin syrup for a sore throat. The lymphoblastic transformation test was positive for erythromycin. Prick tests were negative although the intradermal test was positive at a concentration of erythromycin of 1:10,000. The biopsy showed a perivascular lymphocytic dermatitis. Local treatment with a potent corticoid improved the lesions after 3 days. The Baboon syndrome is uncommon among children. It has a pattern similar to systemic contact dermatitis with particular features (erythema in flexural areas). In our case, the role of erythromycin was documented. However, this antibiotic remains a relatively rare allergen.

Netherton's syndrome: a severe neonatal disease. A case report.

A male infant was born with generalized erythroderma and scaling; the newborn demonstrated poor neonatal development and developed several complications such as hypernatremic dehydration, septicemia, gastroenteritis and seizures. In the neonatal period, the erythema faded, but exfoliation persisted. The parents are healthy but related. One older brother, who died at the age of 3 months, had shown the same clinical picture in the neonatal period and was diagnosed with congenital psoriasis. All clinical investigations, including serum immunoglobulins, complement levels and lymphocyte counts, were normal. Only raised total IgE and multiple positive specific IgE reactions were noted. Skin biopsy revealed an image of ichthyosis. Polarization microscopy of scalp hair showed trichorrhexis nodosa and discrete focal twisting of the hair shaft. This clinical picture and all histological findings are compatible with the indications of Netherton's syndrome. The purpose of this report is to call attention to this severe presentation of congenital ichthyosis in the neonatal period and to the difficulty of a correct diagnosis when confronted with congenital erythroderma.

Eosinophilic pustular folliculitis in three atopic children with hypersensitivity to Dermatophagoides pteronyssinus.

Three children will be described who present recurrent episodes of pruritic papulopustular follicular lesions on the face, the extremities and the trunk. The episodes lasted for 1-3 months with intermittent remission. Each flare was accompanied by hypereosinophilia and an increased total IgE titer. RAST and prick tests were positive for Dermatophagoides pteronyssinus (DPT). Laboratory tests disclosed no infectious or parasitic etiology. Histological examination showed eosinophilic pustular folliculitis (EPF) in each of the 3 cases. The lesions responded well to topical corticosteroids. The aim of this article is to underline the importance of hypersensitivity reactions (in these particular cases to DPT) in the pathogenesis of EPF.