[Protoporhyrinemic photodermatosis simulating hydroa aestivalia].
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Biomedical subjects
Publications and source records attributed to G Burg.
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With biopsies from 101 cutaneous lymphoma patients, enzymecytochemical and immunologic features of the lesions were outlined. Their histo- and cytomorphology and Kiel classification were defined.
Granulomatous slack skin is an uncommon cutaneous T-helper cell lymphoma closely related to mycosis fungoides. To the best of our knowledge this disease has not been previously described in children. We report on an 11-year-old boy who presented with painless slack skin masses in the neck, right axilla and arm, anterior wall of the abdomen, both inguinal regions, and the malleolar and dorsal aspects of the feet. The disease started 3 years earlier with erythematous lesions on the neck and wrists. Histologic examination of a specimen from the abdominal mass revealed an extensive lymphoid infiltrate with scattered multinucleated giant cells extending from the papillary dermis to the subcutis. The lymphoid cells showed the following immunophenotype: CD43+ (MT1), CD45+, CD45RO+, CD20-. The phenotype of the giant cells was lysozyme positive, CD68+ and Mac387-. The tumoral lymphoid cells had clonal rearrangement for the gene of the beta chain of the T-cell receptor (C beta TCR). The disease could be controlled with systemic glucocorticoids. Due to the presence of many histiocytes arranged in aggregates in the papillary and mid-dermis, this case was initially considered to be a cutaneous form of histiocytosis. We recommend deep and extensive biopsies in patients with slack skin disease.
Focal hyperhidrosis of axillae, palms or soles is a frequent, socially debilitating condition triggered by various emotional stimuli. There are several treatment options such as local application of metal sales (aluminum chloride) or tap water iontophoresis, which provide temporary relief for some patients. More recently, local intradermal injections of botulinum toxin A (BTX-A), a neurotoxin blocking the cholinergic stimulus of eccrine sweat glands, offers an effective treatment option with few side-effects. Patient satisfaction rates are high, although treatment effects only last a few months. For definite care, surgical procedures have to be considered.
Based on our own observations in 40 patients and a review of the literature the clinical and histological aspects of dermatitis caused by gold therapy are discussed. There is a broad spectrum of cutaneous side effects following gold therapy. In most of the patients a nonspecific dermatitis can be observed. The skin lesions may also mimic lichen planus, pityriasis rosea or various forms of eczema, obscuring early diagnosis. Histologically four types of reactions can be differentiated including a dermatitis type, vasculitis type, a lichenoid and an urticarial type.
Urticarial vasculitis is a syndrome characterized by a chronic intermittent urticaria and the histological features of necrotizing vasculitis. The skin lesions are often accompanied by arthritis or arthralgia and other systemic diseases. Depressed serum complement can be found in half of the patients.