[Atypical lichen planus in large patches simulating parakeratosis].
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The case of a 46-year-old woman with poikiloderma vasculare atrophicans is discussed. It is a rare clinical form of patch-stage mycosis fungoides characterized by generalized poikiloderma, atrophy, mottled dyspigmentation, and telangiectases.
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In 1937, Sulzberger and Garbe singled out an exudative discoid and lichenoid chronic dermatosis characterized by the combination of various symptoms which by themselves are not specific from the heterogeneous eczema group. The report of a 7-year-old girl is used as a basis to describe the characteristics of the disease and to present the authors' own interpretation. Clinically, there were discoid and lichenoid lesions with severe pruritus. Blood examination revealed eosinophilia. Histopathological examination of skin lesions showed psoriasiform, spongiotic, lichenoid dermatitis. A therapeutic regimen of oral corticosteroids led to complete regression of the skin changes. We feel that there are no clinical or histological findings to differentiate Sulzberger-Garbe disease definitely from extensive nummular eczema.
Erythrokeratodermia figurata variabilis (EKV) is a rare disorder of cornification inherited as an autosomal dominant trait. Genetic linkage to the Rh locus on chromosome 1 has been recently documented. In 1957, Sommacal and Schnyder reported on a family with 14 affected members. We have reexamined this pedigree, which counts 77 members with 29 affected persons over five generations (45 females, 31 males). Twenty females and 9 males were affected. In all patients EKV presented in the first year of life, and several mothers noted the erythematous lesions at birth of their children. The hyperkeratotic lesions appeared later. The reddish macules changed within hours to days. The erythematous areas were sharply outlined and sometimes surrounded by an anemic border. Only few members stated that their erythema could persist for more than a week. Clear triggers were emotional stress and changes of temperature. In all but two of the patients erythema was prominent and in the others hyperkeratotic lesions were more severe. Most patients had a burning sensation in their red areas. There was a marked tendency for improvement of EKV after puberty. Five females reported regular superficial skin peeling on hands and feet. The features in these patients had some similarities with erythrokeratolysis hiemalis.
Ampicillin is the most commonly prescribed antibiotic in the United States, and causes skin reactions in five to ten percent of patient populations. These reactions are considerably more frequent in patients with a viral illness, infectious mononucleosis, and lymphocytic leukemia. Skin reactions to ampicillin are usually of two types: a maculopapular rash in about two thirds of cases, and urticaria in about one third of cases. There is strong evidence that the maculopapular rash is a benign, nonallergic phenomenon. Patients with the maculopapular ampicillin rash are often incorrectly labeled as allergic to ampicillin/penicillin. Ampicillin can be continued and administered again in the future in these patients, and this kind of skin reaction resolves spontaneously in a few days without sequelae. Skin tests are neither required nor recommended to document the nonallergic basis of the maculopapular ampicillin rash.
Plasma concentrations of interleukin-10 (IL-10) were examined in 126 patients with drug-induced cutaneous reactions: maculopapular eruptions (ME), erythema multiforme (EM), erythema multiforme coexisting with erythema nodosum (EMN), drug-induced urticaria (DU), hyperergic vasculitis (HV), Stevens-Johnson syndrome and toxic epidermal necrolysis (SJS/TEN). Activity of the cytokine was measured using the immunoenzymatic ELISA method: a) in the acute stage of disease before treatment was administered, and b) after clearing of skin lesions, after treatment. In the acute stage of disease highly elevated mean concentrations of IL-10 in all 6 groups of patients were found (p<0.001) in comparison with the control. After clearing of clinical symptoms IL-10 concentrations were decreased highly significantly (ME, EM, DU, HV) or significantly (EMN, SJS/TEN) in comparison with the values before treatment, but remained still considerably elevated (p<0.001; p<0.01) when compared with the healthy control. Results of this study indicate that the compensatory antiinflammatory response, expressed as elevated IL-10 activity, is induced as early as in the acute stage of skin lesions and lasts longer than clinical symptoms of drug-induced cutaneous reactions.
Plasma concentrations of interleukin-2 (IL-2) and its soluble receptor (sIL-2R) were examined in 126 patients with drug-induced skin reactions: maculopapular eruptions (ME), erythema multiforme (EM), erythema multiforme coexisting with erythema nodosum (EMN), drug-induced urticaria (DU), hyperergic vasculitis (HV), Stevens-Johnsson syndrome and toxic epidermal necrolysis (SJS/TEN). The activity of both proteins were measured using immunoenzymatic ELISA method: a) in the acute stage of disease, before treatment was administered, and b) after clearing of skin symptoms, after treatment. In the acute stage of disease highly elevated mean concentrations of IL-2 and sIL-2R in all 6 groups of patients were found (p<0.001) in comparison with the control. After clearing of skin lesions IL-2 mean concentrations were lowered to the level not different significantly from the control (p>0.05), but slL-2R mean plasma concentrations, despite the deep decrease, were still highly significantly elevated in comparison with control values (p<0.001).
Plasma concentration of TNF-alpha and its type I receptor (p55TNF-R) was examined in 126 patients with drug-induced skin reactions using immunoenzymatic ELISA method. Patients were subdivided into 6 groups: maculopapular eruptions (ME), erythema multiforme (EM), erythema multiforme coexisting with erythema nodosum (EMN), hyperergic vasculitis (HV), Stevens-Johnson syndrome and toxic epidermal necrolysis (SJS/TEN). In the acute clinical stage highly significant (p<0.001) or significant (p<0.01) elevation of mean plasma concentrations of the cytokine and its receptor was found in all examined groups in comparison with the control. Clearing of clinical symptoms was connected with considerable decrease (p<0.001, p<0.01) of mean plasma levels of the both proteins in comparison with the before treatment values. TNF-alpha concentrations still remained significantly more elevated than those observed in the control. The results indicate that plasma activity of TNF-alpha and its p55 receptor change with the clinical course of the examined drug-induced skin reactions, which suggests the partake of both proteins in the pathogenesis of these diseases.
Inverse psoriasis, rare in clinical practice, refers to psoriasis only or mainly occurring at flexural sites, such as the axilla, antecubital fossae, popliteal fossae, and inguinal creases. It is also known as flexural psoriasis. With a total collection of psoriatic cases from September 2002 to December 2003 at Xijing hospital, we made a retrospective analysis of the disease history, clinical characteristics, and treatment of the patients affected with inverse psoriasis. The results showed that the major clinical manifestations of inverse psoriasis were sharply demarcated erythematous plaques with varying degrees of infiltration and minimal or no scales. Affected areas often involve the groin, axilla, genitals, and umbilicus. The disease responds well to the narrow band UVB therapy. Compared with common psoriasis, inverse psoriasis has similar and unique characteristics in terms of the affected areas, clinical symptoms, and responses to the treatment.
The onset of psoriasis is observed before the age of 10 years in 15% of all patients. The clinical pattern often takes on a peculiar form. Psoriasis guttata--or less frequently nummular psoriasis--is the initial phase during childhood. It is very difficult to establish a diagnosis on the basis of incipient features when childhood psoriasis is located on the head, palms, soles, or on the fingers, toes and nails. Intertriginous or flexural psoriasis, psoriasis spinulosa and oral psoriasis is also described. The onset of napkin psoriasis starts at the age of 3 months. Occasionally, Leiner's disease may develop into a typical, chronic psoriasis. We have concentrated our studies on the less common features of childhood psoriasis: the generalized pustular infantile form, congenital psoriatic erythroderma, acquired psoriatic erythroderma, and infantile arthropathic psoriasis.