A child with a localized hair abnormality. Woolly hair nevus.
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Biomedical subjects
Publications and source records attributed to S Halevy.
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Esophageal involvement in pemphigus vulgaris is rare, found more in women than in men, and can be the only manifestation of pemphigus. Esophageal involvement had been reported only among patients with pemphigus vulgaris and not in patients with other forms of pemphigus. We describe a case of pemphigus vulgaris in which the esophagus was severely involved with only few oral lesions and no involvement of the skin or oropharynx.
A rare case of a young Caucasian female with hypopigmented mycosis fungoides is described. We reviewed and discussed the literature.
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Acute haemorrhagic oedema of infancy (AHEI) is an acute leucocytoclastic vasculitis affecting infants and young children. It has a striking appearance of large purpuric skin lesions in a target-like pattern and marked oedema mainly on the face, auricles and extremities. In some patients there is mucosal involvement as well. We present five patients with skin lesions characteristic of AHEI with no visceral involvement and complete resolution within 7-14 days. In three of our five cases, histopathological examination was performed, and demonstrated typical leucocytoclastic vasculitis. Although sometimes confused with Schönlein-Henoch purpura, we suggest that AHEI should be regarded as a separate entity. Clinical criteria for diagnosis are proposed.
Condyloma acuminatum is a skin infection affecting the anogenital area caused by the human papilloma virus and is usually sexually transmitted in adults. The disease in children is rare and the mode of transmission is controversial. Because of the possibility that children with condyloma acuminatum have been sexually abused, a thorough medical and social evaluation is essential. However, the physician should bear in mind the possibility of nonsexual transmission. Effective management of condyloma acuminatum in prepubertal children involves a multidisciplinary approach and cooperation. We present an 11-month-old girl with genital lesions of condyloma acuminatum. Similar lesions were found in her father's penile area. These findings suggested the possibility of sexual abuse as the cause of the infection. However, detailed, meticulous investigation of the case did not support this suspicion. Our final impression was that transmission was probably nonsexual, as there was no evidence for sexual abuse.
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A classical case of Werner's syndrome is described. In addition to the numerous skin changes that are typically associated with Werner's syndrome, our patients also displayed diffuse lentiginosis, and several of the clinical features of leopard syndrome. Histopathological and ultrastructural findings from a hyperpigmented macule displayed the typical features of a simple lentigo. A striking feature was the presence of melanosomes in Langerhans cells as has been reported in the leopard syndrome. A possible generalized mesodermal defect has been suggested in Werner's syndrome, while the basic defect in the leopard syndrome is thought to be of neuroectodermal origin with pleiotropic changes in the organs derived from the mesoderm. Our patient, with incomplete leopard syndrome and typical Werner's syndrome, may be an example of an association of genetic defects affecting both tissues of neuroectodermal and mesodermal origin.
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A patient who showed a disseminated pustular eruption compatible with pustular vasculitis and concomitant bullous lesions with the features of superficial bullous pyoderma gangrenosum during an exacerbation of ulcerative colitis is described. These clinical findings could be an expression of a common immunopathogenic mechanism of inflammatory bowel disease and the neutrophilic dermatoses. It is suggested that pustular vasculitis is another skin manifestation of ulcerative colitis.
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A case of gamma/delta-positive T-cell lymphoma localized primarily in the subcutaneous adipose tissue and associated with an extensive hemophagocytic syndrome and a rapid, fatal outcome is presented. Previous reports of gamma/delta cutaneous and subcutaneous T-cell lymphomas are reviewed. To define the characteristics of a T-cell subset of neoplastic cells bearing the gamma/delta receptor in a subcutaneous lymphoma, we undertook clinical and laboratory examinations, morphological evaluation of biopsies, electron microscopy, and extensive immunohistochemical studies. Immunohistochemical studies showed that the neoplastic cells expressed the gamma/delta T-cell receptor (CD3+, C gamma M1+) and were CD2+, CD43+, CD45+, CD45RO+, PCNA+, but were beta F1-, CD1-, CD4-, CD8-, CD15-, CD20-, CD25-, CD30-, CD45R-, CD57-, CD68-, Mac 387-, and HLA Dr-. To the best of our knowledge, this is the first report of a subcutaneous T-cell lymphoma of gamma/delta type involving the subcutaneous tissue and associated with a hemophagocytic syndrome.