1/86. Hereditary perioral pigmented follicular atrophoderma associated with milia and epidermoid cysts.Eight members of a single family all presented the characteristic changes of facial, especially perioral, pigmented follicular atrophoderma, with numerous milia and epidermoid cysts. For this condition. diagnosis at a glance may be possible because of the perioral cutaneous manifestations. Histopathological examination of follicular atrophoderma revealed proliferation of basaloid cells continuous with the epidermis and coarse collagen fibres, with a decreased density of elastic fibres around the basaloid cells. Two of the eight individuals also showed generalized hypohidrosis. The eight affected persons were the proband, her son, mother, uncle, two younger sisters, cousin and nephew: an autosomal dominant mode of transmission was suggested from this family tree. The patients' symptoms resembled those of Bazex-Dupre-Christol syndrome, except for the different distribution of the follicular atrophoderma and the absence of basal cell carcinoma and hypotrichosis. This disease may be an entirely new syndrome characterized by perioral pigmented follicular atrophoderma associated with milia and epidermoid cysts.- - - - - - - - - - ranking = 1keywords = family, member, life (Clic here for more details about this article) |
2/86. Epstein-Barr virus-associated lymphoproliferative skin lesion with recurrent necrotic papulovesicles of the face.A 24-year-old man showed recurrent necrotic papulovesicles of the face. The cutaneous lesions started with erythema, followed by vesicles, necrosis, and crusting, and leaving depressed scars. light avoidance did not prevent the development of the lesions. Histologic examination revealed lymphoproliferative lesions confined to the skin. Latent Epstein-Barr virus (EBV) infection was detected in the lymphoid cells from the skin lesions by in situ hybridization. A moderate to high dosage of corticosteroids suppressed the development of new skin lesions, but relapses occurred when these systemic corticosteroids were tapered.- - - - - - - - - - ranking = 0.0092653466057579keywords = life (Clic here for more details about this article) |
3/86. Giant cutaneous horn associated with verruca vulgaris.Cutaneous horn (cornu cutaneum) is the clinical description of a hyperproliferation of compact keratin in response to a wide array of underlying benign and malignant pathologic changes. We report a patient with a giant cutaneous horn associated with a verruca vulgaris. The possible causes of cutaneous horns are reviewed.- - - - - - - - - - ranking = 0.0018530693211516keywords = life (Clic here for more details about this article) |
4/86. Sinus histiocytosis (Rosai-Dorfman disease) clinically limited to the skin.Sinus histiocytosis with massive lymphadenopathy (SHML) is an idiopathic proliferation of unique histiocytes that have vesicular nuclei and voluminous pale cytoplasm, often with emperipolesis. Pure cutaneous involvement is very rare. We describe a patient with SHML limited to the skin whose lesion has spontaneously regressed. A 35-year-old Korean male visited the Department of dermatology due to facial rash for 2 months. A 3 x 3.5 cm-sized well-demarcated dark erythematous nontender plaque was noted on the right cheek. skin biopsy showed dense, nodular infiltrates of histiocytes with abundant cytoplasm and vesicular nuclei rimmed by lymphoplasma cell aggregates throughout the upper and mid-dermis. The histiocytes were immunohistochemically positive for S-100 protein and CD68, but negative for CD1a. Laboratory tests and a thorough physical examination revealed no abnormalities. These findings suggested that this was a case of SHML clinically limited to the skin. The skin lesion was initially resistant to steroid therapy, but began to regress 10 months after the onset without further treatment.- - - - - - - - - - ranking = 0.0018530693211516keywords = life (Clic here for more details about this article) |
5/86. Acquired angioedema with C1 inhibitor deficiency: is the distinction between type I and type II still relevant?BACKGROUND: Acquired angioedemas are divided into type I associated with lymphoproliferation and type II caused by anti-C1-inhibitor antibodies. Recent reports have suggested that this distinction is not so clear-cut, mainly because of the presence of antibodies against the C1 inhibitor in some cases belonging to the type I group. We report herein 2 additional cases of acquired angioedema with anti-C1-inhibitor antibody. MATERIAL AND methods: One man and 1 woman had had acquired angioedema for several years. In the man, a monoclonal component had been detected several years before the present study. In the second patient, a monoclonal component was detected during the study. The following data were studied on successive blood samples collected during angioedema manifestations: complement component levels, functional activity of the classical pathway, functional and antigenic C1 inhibitor doses, ELISA test to detect autoantibodies to C1 inhibitor and Western blot analysis of the C1 inhibitor. RESULTS: In both patients, CH50 and C4 activities were decreased, and an autoantibody to C1 inhibitor was detected. In 1 case, the antibody appeared after the monoclonal component; in the second case, it appeared before and belonged to a different immunoglobulin class. CONCLUSION: Our data suggest that the distinction between type I and type II acquired angioedema is no longer valid because of overlapping in some cases.- - - - - - - - - - ranking = 0.0018530693211516keywords = life (Clic here for more details about this article) |
6/86. Trichodysplasia spinulosa--a newly described folliculocentric viral infection in an immunocompromised host.This is a case report of an immunocompromised individual who presented with progressive alopecia, friable follicular spinous processes, and erythematous, indurated papules. Examination of skin biopsies using light microscopy and immunohistochemistry revealed pathologic changes of the follicular inner root sheath epithelium with dystrophic trichohyaline granules. Electron microscopy of thin sections of tissue revealed intracellular viral particles with a size and appearance consistent with those in the Papovaviridae family. Electron microscopy of negatively stained extract from a homogenized lesion also demonstrated icosahedral viruses with papovavirus morphology. We believe this is a previously unreported folliculocentric viral infection in an immunosuppressed human host and have termed this entity "trichodysplasia spinulosa".- - - - - - - - - - ranking = 0.49244844313534keywords = family (Clic here for more details about this article) |
7/86. Grzybowski's generalized eruptive keratocanthomas: a case report.A 47-year old woman with Grzybowski's generalised eruptive keratoacanthomas is described. There was no history of skin disease in her family, except for an uncle's basal cell carcinoma. From 1995 she developed multiple lesions of various size, ranging from hundreds of small follicular lesions to large typical keratoacanthomas up to 5 cm in diameter, scleroderma-like facial skin and marked ectropion. Histological examination of small and large skin lesions was typical of keratoacanthoma, and no human papillomavirus was detected by polymerase chain reaction. Oral treatment with acitretin had no effect. Both cyclophosphamide and methotrexate therapy were refused by the patient despite the progressive course of the disease. Blepharoplastic surgery had some effect on eye symptoms. The etiology of this rare disease is unknown, but is probably related to some genetic defect.- - - - - - - - - - ranking = 0.49244844313534keywords = family (Clic here for more details about this article) |
8/86. Primary pachydermoperiostosis: a case report.Pachydermoperiostosis (PDP), a rare genodermatosis, occurred in a 38-year-old Indian male. He presented with progressive thickening of the skin on the face and scalp of 15 years duration. Widening of his wrists and ankles and broadening of the fingers and toes had also developed since then. He was born of a consanguineous marriage and had no family history of a similar disorder. He had the typical findings of complete form of PDP including cutis verticis gyrata, coarse facial features, clubbing of the digits in the skin, and periostosis and cortical thickening at the distal ends of long bones of the extremities and small bones of the hands and feet. PDP has two different forms--primary and secondary. These two entities are differentiated by family history and presence or absence of a primary lesion, usually in the lungs. Clinically, in secondary PDP, the cutaneous findings (pachydermia, seborrhoea, oiliness) are less severe than primary PDP; osteoarthropathy is more severe and painful in secondary PDP, especially with congenital heart disease. The present case was suffering from primary PDP that had expressed itself in its complete form.- - - - - - - - - - ranking = 0.98489688627067keywords = family (Clic here for more details about this article) |
9/86. Sebaceous carcinoma arising on actinic keratosis.We report two cases of sebaceous carcinoma arising on actinic keratosis. The first patient, a 75-year-old female, had a granuloma pyogenicum-like tumor on her left temple, and the second patient, an 81-year-old female, developed a tumor with erythema on her left cheek. In both cases, histopathological examination revealed findings typical of sebaceous carcinoma in the center of the tumors, and in the periphery, actinic elastosis and intraepidermal proliferation of squamoid atypical cells without vacuolation was observed. Immunohistochemical examinations using six antibodies also revealed that neoplastic cells of both cases demonstrated sebaceous differentiation. These cases suggest that extraocular sebaceous carcinoma may arise from actinic keratosis.- - - - - - - - - - ranking = 0.0018530693211516keywords = life (Clic here for more details about this article) |
10/86. Erythematous plaques due to platelet plugging: a clue to underlying myeloproliferative disorder.We report a syndrome in a middle-aged woman characterized by tender erythematous plaques with histologic evidence of dramatic dermal vessel occlusion. These cutaneous findings occurred in association with progressive inferior vena cava and portal vein thrombosis while on coumarin anticoagulation, following hepatic transplantation for budd-chiari syndrome. The material occluding dermal vessels was proven by immunohistochemical staining to be platelet plugs. These findings led to the diagnosis of an underlying myeloproliferative disorder explaining both her cutaneous and liver abnormalities and institution of appropriate platelet directed anticoagulation with aspirin.- - - - - - - - - - ranking = 0.0092653466057579keywords = life (Clic here for more details about this article) |
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