ABSTRACT
Langerhans cell histiocytosis is most common in children and is unusual in the elderly. We describe 3 cases of langerhans cell histiocytosis limited to the skin in elderly patients. Biopsy specimens showed a dermal infiltrate abutting the epidermis composed of atypical langerhans cells with abundant eosinophilic cytoplasm and a "kidney-shaped" nucleus. Immunoperoxidase stain CD1a was positive in all 3 cases and S-100 stain was positive in 2. Electron microscopy revealed Birbeck granules in the cytoplasm of the atypical langerhans cells in 2 cases. Langerhans cell histiocytosis with skin involvement has a chronic course with an overall good prognosis. However, cutaneous manifestations may precede systemic involvement by many years.
Subject(s)
Aging/pathology , Histiocytosis, Langerhans-Cell/pathology , Skin Diseases/pathology , Adult , Aged , Aged, 80 and over , Female , Histiocytes/pathology , Histiocytes/ultrastructure , Histiocytosis, Langerhans-Cell/complications , Humans , Male , Middle Aged , Skin Diseases/complicationsSubject(s)
Burns, Electric/therapy , Lip/injuries , Splints , Child, Preschool , Female , Humans , Infant , MaleSubject(s)
Dermatitis Herpetiformis/etiology , Glutens/adverse effects , Adult , Aged , Antibodies/analysis , Dermatitis Herpetiformis/diet therapy , Dermatitis Herpetiformis/immunology , Dermatitis Herpetiformis/pathology , Female , Fluorescent Antibody Technique , Gliadin/immunology , Glutens/administration & dosage , Humans , Immunoglobulin A/analysis , Immunoglobulin G/analysis , Immunoglobulin M/analysis , Intestine, Small/pathology , Male , Middle AgedABSTRACT
Seventy-eight patients with dermatitis herpetiformis have been followed up for periods ranging from 3 to 14 years (mean 7.4). Forty-two patients were treated with gluten-free diet (GFD) and thirty-six took a normal diet (ND). Thirty of the forty-two (71%) taking the GFD were able to discontinue drugs previously needed to control their rash compared with five (14%) of the thirty-six patients taking a ND. The mean time taken to reduce drug requirements for patients taking a GFD was 8 months (range 4-30), and for stopping drugs, 29 months (range 6-108). The incidence of macroscopic abnormality of the small intestine decreased from 69 to 15%, and the mean intra-epithelial lymphocyte count decreased significantly in those patients taking a GFD, whereas there was no significant change in patients taking a ND. The improvement in the skin and intestinal lesions was related to the strictness of the GFD.
Subject(s)
Dermatitis Herpetiformis/diet therapy , Adolescent , Adult , Dapsone/administration & dosage , Dapsone/adverse effects , Dermatitis Herpetiformis/drug therapy , Dermatitis Herpetiformis/pathology , Drug Administration Schedule , Female , Follow-Up Studies , Glutens , Humans , Immunoglobulin A/metabolism , Intestines/pathology , Male , Middle Aged , Skin/immunology , Time FactorsABSTRACT
Eleven patients with dermatitis herpetiformis, all requiring dapsone to control their rash and taking a normal diet, were given disodium cromoglycate (DSCG) 1.5-1.6 g daily. Eight out of the eleven patients continued to take DSCG for periods varying from 6-11 months, the other three patients chose to discontinue the DSCG before 6 months. Of the eight patients taking DSCG for at least 6 months, none was able to stop taking dapsone. In three of the eight, the dapsone requirements were unaltered, whilst in two it decreased and in three it increased. The mean daily dose of dapsone was 105 mg/day before DSCG and 141 mg/day after DSCG. In the eight patients who took DSCG for at least 6 months, intestinal biopsies were performed before and after this drug. The macroscopic appearance was unchanged in four, improved in two and worse in two. The mean interepithelial lymphocyte count was 346 before and 342 after DSG.
