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At least 19 recordsLinked to original sources

Hidrotic ectodermal dysplasia: study of a large Chinese pedigree.

Hidrotic ectodermaldysplasia was found, to our knowledge, for the first time in a Chinese family in Malaysia, and it affected 15 members in five generations. The disease, which is transmitted as a non-sex-linked autosomal dominant trait, presumably originated from southern China. All 15 members had the typical nail, hair, and skin lesions, and we observed three different types of nail defects. Scalp alopeica was more extensive in the female members while keratoderma of the palms and soles was more notable in the male members. The nail and skin lesions also became severer with age. Except for the infectious eczematoid dermatitis present in the propositus, none had other skin or systemic disorders. All were relatively healthy and had normal life expectancies;

Adult

Clinical features of 22 cases with "inter-sterno-costo-clavicular ossification". A new rheumatic syndrome.

We present 22 cases with inter-sterno-costoclavicular ossification. Clinical and pathological findings show that abnormal ossification observed in this situation is due to non-suppurative chronic inflammation of the soft tissues around the sterno-costo-clavicular region such as the costo-clavicular ligament. We have classified X-ray findings into three stages according to the extent of the ossification; localized, generalized, and hyperostotic, and show that the disease progressed in this sequence. A considerable number of the patients showed abnormal X-ray findings in the spine or the sacro-iliac joint. Frequent association of pustulosis palmaris et plantaris was noted in this disease. Most of the cases were treated effectively with anti-inflammatory drugs, but a few cases required surgical resection of the ossified mass with the clavicle or the first rib in order to relieve the severe pain.

Adult

Chronic recurrent multifocal osteomyelitis and pustulosis palmoplantaris.

Based on nine patients 4 to 26 years of age, we describe the clinical characteristics of chronic recurrent multifocal osteomyelitis. This disorder was characterized by an insidious onset of fever, local swelling and pain in affected bones, and radiologic findings suggesting osteomyelitis. The lesions were mainly localized to the clavicles and the metaphyses of tubular bones. The clinical course was characterized by intermittent periods of exacerbation and improvement over several years. Six of the patients had recurrent pustulosis palmaris and plantaris, which closely parallelled the exacerbations of the bone lesions. Biopsies from the bone lesions showed nonspecific inflammatory changes with granulocytic infiltration. Repeated bacterial and fungal cultivations from blood, bone biopsies, and pustules were negative. Immunologic investigation revealed no abnormality common to the patients. There was no indications of a genetic etiology. The pathogenesis of the disorder is unknown. Antibiotic treatment had no obvious effect but corticosteroid therapy appeared to be of benefit in some patients. The long-term prognosis appears to be relatively good.

Adolescent

Volar melanotic macules.

Asymptomatic light brown or tannish-gray macules are seen on palms and/or soles of black patients, and occasionally on the volar surfaces of whites. They may be mistaken for lesions of secondary syphilis or other postinflammatory hyperpigmented dermatoses. Histologic examination of 14 specimens obtained at necropsy showed purely epidermal hyperpigmentation of all epidermal layers; or (3) melanin restricted to large dendritic melanocytes without appreciable transfer to keratinocytes. The number of melanocytes was not significantly increased, and with one exception, there were no nevus cells. These volar melanotic macules have close clinical and histologic resemblance to melanotic macules observed occasionally on the vermillion area of the lips.

Adult

Disseminated superficial actinic porokeratosis. Analysis of an affected family.

A family is analysed in which disseminated superficial actinic porokeratosis (DSAP) occurred in five members. All three children of one generation are affected. Clinical, histopathological and genetic aspects of DSAP are discussed. Disseminated superficial actinic porokeratosis appears to be a clinical variant of porokeratosis of Mibelli. Since DSAP is a genodermatosis inherited as an autosomal dominant it can occur in any geographical location; however excess sunlight can definitely exacerbate the condition.

Adult

DNA synthesis and mitosis in uninvolved epidermis of persistent palmoplantar pustulosis.

Mitotic and DNA synthesizing cell counts have been performed in uninvolved epidermis of twenty-one patients with persistent palmoplantar pustulosis (PPP). There was no difference in mitotic counts and DNA synthesis in PPP compared with normal epidermis, but both were significantly lower than those found in the clinically uninvolved epidermis of patients with psoriasis.

Aged

Oral methoxsalen photochemotherapy of recalcitrant dermatoses of the palms and soles.

PUVA therapy successfully cleared various dermatoses mainly confined to the palms and soles in 18 of 20 patients treated. The conditions treated were: plaque-type psoriasis, pustular psoriasis, endogenous eczema and persistent palmoplantar pustulosis. Seventeen patients were treated in a controlled study of PUVA therapy versus no treatment at all and in 16 of these patients the disease was cleared in the PUVA-treated areas while the untreated areas remained unchanged or deteriorated. Twelve of the 18 patients were maintained in a clear state by continued maintenance PUVA treatment over 6--31 months while 3 patients had a spontaneous remission and are free of disease off all treatment.

Adolescent

Clinical study of porokeratosis: reports of 10 cases.

Morphological and histopathological observations made over 4 years of 10 patients with porokeratosis are described. Absence of familial involvement was notable. Association of porokeratosis with tinea cruris, leprosy and epithelioma was seen, and their possible correlation is discussed.

Adolescent

Cinnamic aldehyde allergy.

Positive patch test reactions to 2% cinnamic aldehyde were obtained from 2.8% of 34 males and 9.1% of 55 females. The 9.1% female reactivity may be traceable to perfumed cosmetics. Simultaneous Jasmin allergy is documented as is the occurrence of alpha-amyl cinnamic aldehyde in synthetic Jasmin. The relevance of these reactions is discussed. In two of the patients a proper diagnosis was not made from the original contact history. Only after a screening tray demonstration of cinnamic aldehyde allergy could a relevant history be taken from these patients.

Acrolein

Generalized eruptive porokeratosis of Mibelli with associated psoriasis.

A case of eruptive porokeratosis of Mibelli with diverse morphologic features, including circinate macular, circinate plaque and verrucous varieties is presented. No matter how variable the clinical presentation may be, the histologic hallmark of porokeratosis, the cornoid lamellae, is always present. The cornoid lamellae vary in height in relation to how prominent the thready ridge of the clinical lesion appears. Our patient also had psoriasis which initially masked the porokeratotic lesions both clinically and histologically. Awareness of the various clinical expressions of porokeratosis of Mibelli would 1) make unnecessary the segregation of certain forms of porokeratosis into separate entities, and 2) help in the recognition of less classical forms of porokeratosis.

Foot Dermatoses

Recalcitrant pustular eruptions of the extremities.

Forty-seven biopsies of pustules from patients with recalcitrant pustular eruptions of the palms and soles were examined blind and the results were subsequently compared among three clinical sub-groups: acrodermatitis continua, pustular psoriasis and pustulosis palmaris et plantaris. With the exception of three cases, it was not possible to make a diagnosis of psoriasis on the basis of histology alone. Histological findings were otherwise uniform and typical: intra-epidermal, unilocular, well-delineated pustules. No significant differences were found among the three subgroups. In view of the uniform histology, the unknown etiology and the uncertain clinical classification, the authors prefer the clearly descriptive term pustulosis palmaris et plantaris.

Acrodermatitis