摘要:

Abstract:We examined skin biopsy specimens from 17 of 19 newborns or infants with generalized ichthyosiform, exfoliative, seborrheic, or psoriasiform erythroderma. The specimens showed similar characteristic but nonspecific and therefore, at first sight, uninformative histologic features. Morphologically, the skin was affected overall with a persistent outbreak of eczema‐like eruptions of subacute or chronic dermatitis. Pronounced dermal inflammatory processes were obvious by their perivascular and interstitial presence as well as exocytosis of lymphocytes, macrophages, and neutrophils. Epidermal barrier function was impaired by the highly suppressed terminal differentiation, with thin or in part completely absent stratum corneum, decrease of keratin filaments, decrease or lack of kera‐tohyalin granules, and of keratinosomes containing stacks of lipid membranes. As a result, the formation and discharge of epidermal barrier lipids from the keratinosomes that normally provide intercellular lamellar sheets at the granular‐horny layer interface contributing to the epidermal barrier, was highly disturbed. The concomitant loss of water, electrolytes, and proteins by fluid exudation caused the patients severe metabolic problems and recurrent infections. The suspicion of Netherton syndrome was eventually confirmed in 18 patients by light microscopic demonstration of bamboo hairs (trichorrhexis invaginata), mostly from the scalp, but also in velius hairs and eyelashes. Atopy actually belongs to the symptom triad defining Netherton syndrome and is, in our opinion, primarily responsible for the pathologic events within the skin and of the keratinizing parts of the growing hair shafts. Differential expression of the atopic condition determines the appearance of the keratinization disorder of the skin, namely, severe, generalized, exfoliative erythroderma or milder forms of ichthyosis linearis circumflexa Comèl. Retinoid treatment seems to be contraindicated in these conditions since their biopharmacologic effects involve suppression of terminal differentiation, which is the proper pathognomonic event. In six patients the condition had a fatal course within months because of hypernatremia, recurrent infections, failure to thrive, and sepsis. Our aim is to call attention to and reaffirm that in congenital or early infantile cases of generalized exfoliative erythroderma, Netherton syndrome should be suspected as the underlying d

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1996.tb01202.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

Abstract:We report 3 children with leukemia cutis observed at the initial diagnosis of systemic leukemia. Leukemia subtypes in the three children were congenital monocytic, acute undifferentiated, and acute monocytic, respectively. The patients were girls age 10 days, 14 years, and 11 months, respectively, at diagnosis. We describe the clinical features of the cases and the results of immunohistochemical studies on paraffin‐embedded skin biopsy specimens. The skin lesions were tumors and areas of reddish purple erythema in the first child, pigmented erythema in the second, and bright red erythema in the third. In the first two patients skin lesion biopsy specimens had dense leukemic infiltrates in the dermis with reactive T lymphocytes scattered among them. In the third patient, the infiltrating cells were almost all reactive T lymphocytes, with a few leukemic cells. A relationship between the leukemic‐reactive cell ratio and the prognosis was suggested; dense leukemic cell infiltrates may be associated with a poor progno

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1996.tb01203.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

Abstract:Clinical and pathologic features of five cases of epidermolysis bullosa simplex, Dowling‐Meara type (EBS‐DM), are described. Four patients were children, and two were related (father and daughter). Clinical history revealed blistering at birth in three patients; in all of them the signs and symptoms improved with age. Histopathologic and ultrastructural examinations showed cytolysis of the basal cells and clumping of the tonofilaments within the cytoplasm of keratinocytes. Two distinct types of clumps were observed: round (3 patients) and whisklike (2 patients). Two patients had both types of clumps. The presence of both types in the same patient suggests that subtyping of the disease is still premat

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1996.tb01204.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

Abstract:Subungual exostosis is typically a benign, acquired tumor of cartilaginous bone occurring on the medial surface of the distal hallux. A 16‐year‐old boy with subungual exostosis of his left great toe is presented. We evaluated the English language literature on this topic from 1857 to 1994; including our patient, 312 cases have been described. The diagnostic characteristics (histologic and radiologic), etiologic features, and therapeutic management were reviewed. Subungual exostosis more commonly affects women than men In a ratio of 2:1. Most lesions occur in the early twenties; however, a minimum of 51 (16%) of the 312 cases of subungual exostoses appeared in children 18 years of age or younger. Seventy percent of lesions occurred on the first hallux. There was frequently an association with trauma. The diagnosis of subungual exostosis may be suspected from the clinical presentation and confirmed with radiographic examination. The treatment is surgi

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1996.tb01205.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

Abstract:Congenital psoriasis without an underlying nevus following the lines of Blaschko is described In a 6‐year‐old girl. In addition to this special type of psoriasis, she suffered from atopic dermatitis. The lesions were aggravated by sun exposure and phototherapy treatments, as well as by upper airways infections, without any clear explanation of these trigger factors. We propose to call this form of psoriasis congenital blaschkoid psoria

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1996.tb01206.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

Abstract:Congenital smooth muscle hamartoma usually manifests as a well‐circumscribed, hyperpigmented plaque, frequently hypertrichotic, on the trunk or extremities. We report such a lesion in a 7‐month‐old girl that presented as a linear, mottled, purplish red plaque appearing In areas to be atrophic, involving her right buttock, posterior thigh and leg, and fifth toe. Although the clinical appearance suggested linear morphea, a biopsy specimen had numerous haphazardly oriented bundles of smooth muscle in the reticular dermis. Masson trichrome staining, smooth muscle specific actin, and electron microscopic studies confirmed the smooth muscle nature of the cells. A diagnosis of smooth muscle hamartoma was made. To our knowledge, this linear clinical presentation has not been described previ

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1996.tb01207.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

Abstract:Herpes zoster in childhood is uncommon even in the setting of known risk factors such as primary varicella zoster infection before 12 months of age and immunocompromised states. We report a 7‐year‐old, otherwise healthy girl with shingles, and review the risk factors, prognosis, and treatment of pediatric zos

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1996.tb01208.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

Abstract:Cutis marmorata telangiectatica congenita (CMTC) is a cutaneous vascular abnormality characterized by a persistent cutis marmorata pattern, spider nevuslike telangiectasia, and superficial ulceration. An infant girl was noted at birth to have characteristic features of CMTC as well as anti‐Ro/SSA antibodies and a positive autonuclear antibody (ANA) test. Serologic studies carried out on her mother were positive for Ro/SSA antibodies as well as ANA (titer 1:2560). Therefore the diagnosis of neonatal lupus was made. We propose that livedo patterns mimicking CMTC might sometimes represent the residual phase of neonatal lupus active during intrauterine lif

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1996.tb01209.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

Abstract:Nevus spilus Is clinically defined as a pigmented skin lesion composed of a light brown macule on which darker, circumscribed macular or papular lesions appear at a later stage. Histologically, nevus cells have been found both in pigmented spots and in the background stain. Cases of transformation into melanoma have been reported in the literature and the excision of these lesions is therefore justified. We discuss surgical techniques used in seven patients with nevus spilus and define their indications with respect to the characteristics of the lesion.

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1996.tb01210.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

Abstract:Topical corticosteroids have been one of the cornerstones of dermatologic treatment for nearly 40 years. At present the most widely used topical steroid ranking system is the vasoconstrictor assay. To determine whether this is a satisfactory measure of the agents’ potency in clinical settings, we examined rankings from the published literature achieved by four different methods: vasoconstriction, clinical outcome, therapeutic index (a ratio of efficacy to systemic safety), and cost, safety, and efficacy. Overall clinical outcome (efficacy) rankings in this study corresponded in only 11 (62%) of 17 topical steroid preparations with the expected vasoconstrictor rankings. The therapeutic index rankings did not correspond with the clinical outcome (33%) or the vasoconstrictor assay (33%) rankings. It was difficult to compare studies because of lack of standardization of clinical trials. We urge that topical steroid rankings not be based solely on vasoconstrictor assays, and that a standard method be developed for clinical trials of the drugs to allow for comparison among preparation

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1996.tb01211.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY