'Zosteriform' lichen planus: is it zosteriform?
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Biomedical subjects
Publications and source records attributed to R Happle.
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The early immune response in alopecia areata is characterized by a Th1 T helper cell cytokine pattern and an aberrant expression of ICAM-1 and HLA-DR molecules on lesional hair bulbs. A counteracting cytokine pattern induced by a therapeutic contact dermatitis is supposed to mediate the hair regrowth. In addition to cytokines, growth factors have been shown to influence immune responses, and we therefore investigated the expression levels for a panel of growth factors in untreated versus alopecia areata after treatment with the contact sensitizer diphenylcyclopropenone. Using semiquantitative reverse transcriptase polymerase chain reaction we detected a striking overexpression of transforming growth factor beta 1 mRNA in successfully treated patients. This cytokine has been shown to be a potent immune response modifier, which can suppress Th1 immune responses. The way in which topical immunotherapy induces hair regrowth in alopecia areata is unknown, but a lesional increased expression of transforming growth factor beta 1 may be a possible mechanism.
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The case of an 81-year-old man with relapsing linear acantholytic dermatosis is described. This is the second description of this disease entity, which is characterized by the histopathologic and ultrastructural features of Hailey-Hailey disease. Clinically, it is characterized by skin lesions that wax and wane in a systematic pattern following the lines of Blaschko.
A clinical entity called "the epidermal nevus syndrome" does not exist. Rather, there are various epidermal nevus syndromes that can be distinguished by clinical, histopathological, and genetic criteria. In this review, five distinct epidermal nevus syndromes, recognizable by different types of associated epithelial nevi, are described. The Schimmelpenning syndrome is characterized by a sebaceous nevus associated with cerebral anomalies, coloboma, and lipodermoid of the conjunctiva. By contrast, cataracts are a prominent feature of the nevus comedonicus syndrome. The pigmented hairy epidermal nevus syndrome includes Becker nevus, ipsilateral hypoplasia of the breast, and skeletal defects such as scoliosis. In the Proteus syndrome, the associated epidermal nevus is of a flat, velvety, nonorganoid type. The CHILD syndrome occurs almost exclusively in girls. The associated CHILD nevus shows unique features such as a diffuse form of lateralization, ptychotropism, and microscopic changes of verruciform xanthoma. The five epidermal nevus syndromes differ in their genetic basis. The Schimmelpenning and nevus comedonicus syndromes are most likely nonhereditary traits. By contrast, the pigmented hairy epidermal nevus syndrome and the Proteus syndrome may be explained by paradominant inheritance. The CHILD syndrome is caused by an X-linked dominant mutation exerting a lethal effect on male embryos. A correct diagnosis of these phenotypes is important for both recognition and treatment of associated anomalies as well as for genetic counseling.
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Antibodies raised against fragments of synthetic peptides of human 5 alpha-reductase isoenzymes 1 (h5 alpha r1) and 2 (h5 alpha r2) were applied to paraffin sections of human skin (scalp, eyelid, lip, breast, scrotum). Immunoreactive sites were differentially distributed, in that h5 alpha r1 immunoreactivity was present in the nuclei of cells in the stratum germinativum (basal and lower portion of the spinous layer) of the epidermis, subepithelial fibroblasts, adipocytes, smooth muscle cells of the scrotal tunica dartos, basal cells of sebaceous glands, excretory duct cells of sweat glands, cells of the dermal papilla and fibrous and outer epithelial sheath of hair roots, as well as endothelial cells of small vessels and Schwann cells of cutaneous myelinated nerves. In contrast, immunoreactivity for h5 alpha r2 was found in the cytoplasm of the cells of the spinous layer (and far less intensely in the basal layer) of the epidermis, subepidermal fibrocytes, and especially in subcutaneous adipocytes. Immunoreactivity was strongest in the non-keratinized portion of the inner epithelial sheath and the cuticle of hair follicles, whereas other portions of the hair root were negative. Sweat glands were stained, whereas sebaceous glands showed only weak diffuse immunoreactivity. In mucocutaneous zones, salivary glands and conjunctival epithelium showed immunoreactive cells. Vascular endothelium displayed immunoreactivity only in the genital region. We present experimental evidence for a differential distribution of 5 alpha-reductase isoenzymes in human skin. This might reflect a diversity in the response of different areas of the skin to androgenic challenge.
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From the study of standard textbooks of dermatology, no comprehensive understanding of the word nevus can be derived. The purpose of this article is to formulate a workable definition. As a first step, various definitions proposed by previous authors are reviewed. Their inconsistency appears to be mainly due to the fact that the term nevus is used to denote both neoplastic and nonneoplastic, congenital and acquired as well as hereditary and nonhereditary skin lesions. Taking this difficulty into account, the author suggests, as a unifying concept, the new category of genetic mosaicism. The following definition is proposed: 'Nevi are visible, circumscribed, long-lasting lesions of the skin or the neighboring mucosa, reflecting genetic mosaicism. With the exception of melanocytic nevi, they do not show neoplastic growth. They never show malignant neoplasia.'--For several types of nevi the concept of genetic mosaicism has already been confirmed at the cellular level. Future cytogenetic and molecular studies should show whether the proposed definition is valid for all other types of nevi and thus generally acceptable.
BACKGROUND: The CHILD syndrome is characterized by an ichthyosiform and inflammatory nevus showing a strikingly unilateral arrangement. No particular name has so far been given to this nevus, which is why this skin disorder has been described under various inappropriate terms such as epidermal nevus, inflammatory linear verrucous epidermal nevus (ILVEN), unilateral ichthyosiform erythroderma, unilateral ichthyosis or verruciform xanthoma. OBJECTIVE: In order to avoid such confusion and to make it easier for clinicians to recognize this skin disease, a new name should be given to this disorder, and the diagnostic criteria should be delineated. METHOD: The term CHILD nevus is proposed and the distinctive clinical, histopathological and ultrastructural features of this disorder are described. RESULTS: A comprehensive clinical and genetic comparison shows that the CHILD nevus can be distinguished from all other types of epidermal nevi by characteristic features such as ptychotropism, waxy yellowish scaling, a unique lateralization pattern showing both diffuse and linear involvement and the presence of foamy histiocytes in the papillae ('verruciform xanthoma'). Contrasting with all other epithelial nevi, the CHILD nevus is an inherited X-linked dominant, male-lethal trait. CONCLUSION: This nevus represents a separate cutaneous entity. Future clinical research will probably show that the underlying gene defect often manifests itself as an isolated skin disorder. Such cases should no longer be confused with ILVEN. Recognition of this particular skin disorder is important for genetic counseling because a woman showing an isolated CHILD nevus has an increased risk of giving birth to a daughter suffering from a complex congenital disorder, the CHILD syndrome.
Cytokines play an important role in both physiology and pathophysiology of human skin, and the possibility that they coordinate the cyclical hair growth cannot be excluded. Several clinical and experimental data point towards interleukin-1 which might be a crucial inducer of hair loss in various hair disease. An aberrant expression of interleukin-1 beta was detected in affected areas of the scalp in alopecia areata. On the other hand, interleukin-1 has been shown to be a potent inhibitor of human hair growth in vitro. Mice showing an overexpression of the interleukin-1 alpha gene develop patchy hair loss. Taking all of the presently available data together, we hypothesize that interleukin-1 is a crucial mediator inducing cessation of hair growth.
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The new term "paraptic eczema" is proposed for a well-defined type of eczema that is elicited by the haematogenous action of an antigen that has already initiated a sensitization of the T-cell-mediated type by topical action on the skin. The term is composed of the Greek words "para" = " beyond, beside" and hapsis = "contact". Many other names have been used in the past to describe partial aspects of paraptic eczema, such as "haematogenous contact eczema", "dyshidrotic eczema", "pompholyx", "dermatophytid", or "tylotic palmar eczema", but these terms are inappropriate for a workable delineation of this nosological entity.
Many different congenital and acquired skin diseases are seen along Blaschko's lines. In 1992, Moulin et al. [40] described five patients affected with a new clinical entity characterized by acquired atrophic band-like skin lesions showing hyperpigmentation. We report on a further patient with this skin disease. Characteristic features are hyperpigmented atrophoderma arranged in a pattern following the lines of Blaschko, with no preceding inflammation and no subsequent induration or scleroderma. The lesions usually appear during childhood or adolescence, but sometimes in young adults. For this skin disease we propose the term 'linear atrophoderma of Moulin'.