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Biomedical subjects

U Sass

Publications and source records attributed to U Sass.

17 recordsLinked to original sources

[Acquired lymphangiectasias or mammary dermolytic blisters with spontaneous resolution].

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.

Aged↗

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.

Basal Cell Carcinoma↗

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.

Aged↗

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.

Diagnosis, Differential↗

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.

Carcinoma, Squamous Cell↗

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.

Anti-Bacterial Agents↗

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.

Consanguinity↗

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.

Adolescent↗

Lymphomatoid papulosis: a clinical case.

We report a case of lymphomatoid papulosis in a 36-year-old man who presented recurrent necrotic skin nodules that regressed spontaneously leaving scars. The condition had persisted since 1988. These nodules were always solitary and located on the extremities. Histological examination revealed a perivascular and interstitial, superficial and deep infiltrate with numerous atypical lymphocytes (> 40%) characterized by cerebriform mononuclear cells and large atypical cells stained by MT1, UCHL1 and Ber-H2 (anti-CD30). No associated disease was found. The patient has been free of lesions for more than a year without treatment. A nosological and physiopathological hypothesis of lymphomatoid papulosis is discussed.

Adult↗

[Plexiform fibrohistiocytic tumor].

Plexiform fibrohistiocytic tumour has been recently described by Enzinger and Zhang. This is a rare tumour with low grade malignancy which requires large excision. We report two cases, one occurring in a 58-year-old man, presenting a voluminous painless mass of 5 cm on his hand, the other occurring in a 9-year-old boy consulting for a nodule on the chest. On histological examination this dermal hypodermal tumour is characterized by a proliferation of histiocytic-like cells grouped in nodules, where they are associated with multinucleated osteoclastic-like cells and a proliferation of fibroblastic-like cells grouped in fascicles, anastomosing in a plexiform pattern. Immunohistochemistry and ultrastructural aspects are described. Differential diagnosis and histogenesis are discussed.

Child↗

Chronic arsenicism: criminal poisoning or drug-intoxication? Report of two cases.

We report two cases with chronic arsenicism. The first one is a young 37-year-old woman who presents leucomelanoderma, plantar keratoderma, polyneuropathy of the legs and transversal striae of the nails. After investigations, criminal intoxication with arsenic caused by her own sister was discovered. The second case is a 42-year-old man who had developed plantar keratoderma, arsenical keratoses and two squamous cell epitheliomas 10 years after a 2-year treatment with Fowler's solution for androgenetic alopecia.

Adult↗

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.

Albinism, Oculocutaneous↗