ABSTRACT
Hydroa vacciniforme is a rare, chronic photodermatosis with onset in childhood. Multiple exposures to UV-A reproduced the symptoms and the vesicular and scarring lesions typical of the sun-induced disease in our patient. Treatment with hydroxychloroquine sulfate, but not indomethacin, reduced the photosensitivity both to sunlight and to artificial UV-A light. Serum complement levels were low while the disease was active and returned to normal after treatment of the patient with hydroxychloroquine.
Subject(s)
Hydroa Vacciniforme/pathology , Adolescent , Complement C3/deficiency , Humans , Hydroa Vacciniforme/diagnosis , Hydroa Vacciniforme/drug therapy , Hydroxychloroquine/therapeutic use , Male , Skin Tests , Ultraviolet Rays/adverse effectsABSTRACT
Eosinophilic fasciitis (EF, a diffuse fasciitis with eosinophilia) is a recently recognized connective tissue disorder. It consists of deeply indurated, bound-down plaques of skin and subcutaneous tissue, most commonly present on the extremities. It is associated with peripheral eosinophilia, hypergammaglobulinaemia and an elevated sedimentation rate. There is usually no evidence of Raynaud's phenomenon, acral sclerosis or visceral involvement. Antinuclear antibodies are usually absent. The abnormal histopathology primarily involves the lower subcutis and the fascia. The clinical and laboratory features usually improve following the administration of systemic corticosteroids. Serious haematological abnormalities have been associated with eosinophilic fasciitis (EF) and have been discussed in detail. The similarities and distinctions between eosinophilic fasciitis and scleroderma have been discussed.
Subject(s)
Eosinophilia/diagnosis , Fasciitis/diagnosis , Adrenal Cortex Hormones/therapeutic use , Biopsy , Blood Sedimentation , Diagnosis, Differential , Eosinophilia/complications , Eosinophilia/drug therapy , Fascia/pathology , Fasciitis/complications , Fasciitis/drug therapy , Hematologic Diseases/complications , Humans , Joint Diseases/complications , Skin Diseases/etiologyABSTRACT
The cutaneous signs of diabetes have been reviewed in the past. This review shpeds new light on old associations. Some skin signs of diabetes are questioned and additional cutaneous manifestations of diabetes are described herein.
Subject(s)
Diabetes Complications , Skin Diseases/etiology , Arteriosclerosis/etiology , Carotenoids/blood , Darier Disease/etiology , Diabetic Angiopathies/etiology , Diabetic Neuropathies/etiology , Granuloma/etiology , Humans , Lipodystrophy/etiology , Necrobiosis Lipoidica/etiology , Pigmentation Disorders/etiology , Skin Diseases, Vesiculobullous/etiology , Xanthomatosis/etiologyABSTRACT
Dyskeratosis congenita is a rare X-linked recessive disease, characterized by mucosal leukokeratosis, nail dystrophy, telangiectasia, reticulated hyperpigmentation, pancytopenia, and a heightened susceptibility to infection and malignancy. We exposed cultured fibroblasts and peripheral leukocytes from normal persons and from 2 unrelated young adult men with dyskeratosis congenita to 4,5',8-trimethylpsoralen and ultraviolet light. We than compared certain of their responses. Labeled DNA from fibroblasts exposed to 4,5',8-trimethylpsoralen and ultraviolet light showed fast-sedimenting DNA, a pattern we interpreted as evidence that cross-linking, psoralen-DNA photoadducts had been formed by the treatment. Fast-sedimenting DNA persisted for 24 hr in dyskeratosis congenita cells but disappeared from normal cells during a 24-hr repair period. Cultured peripheral blood leukocytes from persons with this syndrome similarly exposed to 4,5',8-trimethylpsoralen and ultraviolet light developed more sister chromatid exchanges than did cells from normal persons. These data suggest that a heightened susceptibility in DNA cross-links may be of fundamental importance in the etiology of dyskeratosis congenita.
Subject(s)
Skin Diseases/congenital , Adolescent , Adult , Cells, Cultured , Chromatids/drug effects , Chromatids/radiation effects , DNA , Dose-Response Relationship, Drug , Dose-Response Relationship, Radiation , Female , Fibroblasts/drug effects , Fibroblasts/radiation effects , Humans , Leukocytes/drug effects , Leukocytes/radiation effects , Male , Photochemotherapy , Skin Diseases/blood , Skin Diseases/pathology , Trioxsalen/pharmacology , Ultraviolet RaysSubject(s)
Anemia, Aplastic/immunology , Antibodies/immunology , Fascia , Anemia, Aplastic/pathology , Bone Marrow/immunology , Colony-Forming Units Assay , Fascia/pathology , Hematopoiesis , Humans , Inflammation/immunology , Inflammation/pathology , Lymphoproliferative Disorders/complications , Lymphoproliferative Disorders/immunology , Male , Middle AgedABSTRACT
An awareness of the cutaneous signs of diseases of the pancreas is extremely valuable. They alert the astute examiner to several life-threatening problems that result from both benign and malignant islet cell tumors, adenocarcinomas of the pancreas, and pancreatic endocrine and inflammatory diseases. Many of the cutaneous manifestations of pancreatic disease are discussed in detail, with emphasis on both the clinical presentation and pathogenesis.
Subject(s)
Pancreatic Diseases/complications , Skin Manifestations , Adenoma, Islet Cell/complications , Dermatitis/etiology , Fat Necrosis/etiology , Glucagon/metabolism , Humans , Pancreatic Neoplasms/complications , Pancreatic Neoplasms/metabolism , Pancreatitis/complications , Panniculitis, Nodular Nonsuppurative/diagnosis , Pigmentation Disorders/etiology , SyndromeABSTRACT
An awareness of various dermatoses which reflect internal malignancy will facilitate the physician's ability to recognize or even anticipate malignant disease. There have been several previous reports on this subject. This review emphasizes the nonspecific cutaneous changes associated with malignancy. New skin markers of cancer are also discussed.
Subject(s)
Neoplasms/diagnosis , Skin Manifestations , Acanthosis Nigricans/etiology , Breast Neoplasms/complications , Carcinoma, Bronchogenic/complications , Carcinoma, Squamous Cell/complications , Dermatomyositis/etiology , Erythema Multiforme/etiology , Esophageal Neoplasms/complications , Female , Granuloma/etiology , Herpes Zoster/etiology , Humans , Ichthyosis/etiology , Keratoderma, Palmoplantar/etiology , Lung Neoplasms/complications , Malignant Carcinoid Syndrome/complications , Neoplasm Metastasis , Neoplasms/complications , Ovarian Neoplasms/complications , Paget Disease, Extramammary/complications , Pancreatic Neoplasms/complications , Thrombophlebitis/etiologyABSTRACT
A method is presented for the separation of epidermal strata by the successive elimination of either the basal or basal and spinous cells with 0.24 M NH4Cl at pH 9.5. Histologic evidence suggests that the residual epidermal strata obtained after incubation of the skin with NH4Cl are reproducible; hence, this technique circumvents loss of granular layer histidine-rich protein inherent with trypsin separation and provides an effective procedure for biochemical analysis of arginine-rich and lysine-rich proteins in the various differentiating epidermal cells.