Localized scleroderma with cutaneous calcinosis. A distinctive variant.

Two patients had a distinctive variant of localized scleroderma. Both have a history of sclerodermatous changes of the skin over the face developing relatively late in life and accompanied by hair loss, cutaneous calcification, and prominent beaking of the nose. A striking lack of systemic involvement also was noted.

Eczema herpeticum caused by herpesvirus type 2. A case in a patient with Darier disease.

This, to our knowledge, is the first documented report of eczema herpeticum due to infection with herpesvirus hominis type 2. The virus was isolated from a patient with known Darier disease who developed Kaposi varicelliform eruption. Recent increases in the incidence of infection with type 2 herpes simplex as well as the high frequency of recurrence with this virus make this infection of potential significance in patients with preexisting cutaneous disease.

Management of necrotizing vasculitis with colchicine. Improvement in patients with cutaneous lesions and Behcet's syndrome.

Six patients with necrotizing vasculitis were treated with oral colchicine as part of an open study. Four patients with cutaneous vasculitis and normal levels of serum complement and one patient with vasculitis associated with Behcet's syndrome demonstrated clinical improvement while receiving colchicine. One patient with cryoglobulinemia, hypocomplementemia, and cutaneous vasculitis showed no response to colchicine therapy. In three patients, clinical improvement persisted after its withdrawal. Colchicine may be effective in controlling cutaneous necrotizing vasculitis and Behcet's syndrome through its effect on polymorphnuclear leukocyte function.

Premature cataracts in a family with hidrotic ectodermal dysplasia.

In a family with hidrotic ectodermal dysplasia affecting five members in three generations, bilateral premature cataracts have developed in four of the five affected individuals. To our knowledge, this represents the first report of a family in which bilateral premature cataracts appear to be inherited with hidrotic ectodermal dysplasia.

Acute febrile neutrophilic dermatosis in children. Report of two cases in male infants.

Two male infants manifested a complex of symptoms and signs that we believed represented a distinctive hypersensitivity disease, with features of acute febrile neutrophilic dermatosis and allergic vasculitis. Both were less than 1 year of age, and both acquired their disease during or after an upper respiratory illness. Each infant was initially seen with waxy and erythematous to slightly violaceous papules that enlarged to form plaques. The lesions developed at sites of trauma and were distributed on the trunk, face, and extremities. Both infants were febrile and had leukocytosis. On histopathologic examination, biopsy specimens taken from the lesions demonstrated a pronounced dermal infiltration of polymorphonuclear leukocytes. These lesions responded to treatment with prednisone and topical fluorinated corticosteroids, healed with scarring, and recurred after initial improvement.

Angiolymphoid hyperplasia with eosinophilia: removal with carbon dioxide laser in a patient on chronic oral anticoagulants.

A patient with angiolymphoid hyperplasia with eosinophilia of the arm is described. In spite of anticoagulant therapy, successful surgical removal of the lesion was carried out with carbon dioxide laser excision and vaporization.

Management of lacerations in sports: use of a biosynthetic dressing during competitive wrestling.

Lacerations occur commonly during competitive contact sports. Such injuries often limit the ability of the athlete to continue competition because of concerns about further trauma to the site and risks of infection. We describe herein the use of a biosynthetic dressing, Duoderm Thin, to protect lacerations received during competitive wrestling. The dressing was able to support the skin, protect the laceration from further injury, shield the wound from exposure to infectious agents, and prevent transmission of blood or serum to other wrestlers. Such protection enabled two wrestlers to continue competition and/or practice without adverse effects.

Skin cancer awareness programs: success of a statewide program of education and screening in Ohio.

Skin cancer has been increasingly identified as an important public health concern. Dramatic increases in the incidence of skin malignancies have led the dermatologic community in Ohio to organize a statewide program of education and free public screenings. The program reached multiple geographic areas throughout Ohio. Educational programs were presented on television and radio, in newspapers, in independent programs, and in schools. Skin cancer screenings ultimately evaluated 5,492 patients. Six hundred and twenty-three patients were found to have suspected malignancies, while an additional 1,647 patients were found to have premalignant lesions of skin. A total of 2,370 patients were therefore found to have significant findings (43.2%). These results emphasize the need for public education concerning this condition.

The superficial mycoses and the dermatophytes.

More than 100,000 species of fungi have been identified worldwide, but only a few dozen are of medical significance. Because of the opportunistic nature of these organisms and the prevalence of immunodeficient conditions, the number of medically important fungi is increasing. Identification of the causative organism is of value in the choice of correct therapy. The development of new antifungal drugs has made it increasingly important to be able to distinguish one fungus from another. This article reviews the laboratory diagnosis of the fungi generally classified as the superficial mycoses and the dermatophytes.

Hodgkin's disease and mycosis fungoides in a married couple.

Hodgkin's disease and mycosis fungoides are malignancies in which there is abnormal T lymphocyte proliferation. The authors report the occurrence of Hodgkin's disease and mycosis fungoides in a husband and wife, respectively. This represents another instance of clustering of cases of T lymphocyte malignancies.

Basal cell nevus syndrome. Unresponsiveness of early cutaneous lesions to topical 5-fluorouracil or dinitrochlorobenzene.

A single patient with basal cell nevus syndrome is presented. Prolonged application of 5% 5-fluorouracil and dinitrochlorobenzene was unsuccessful in inducing regression or clearing of his cutaneous lesions. Our results suggest that these therapies, at least for the nevoid-appearing lesions, may not be effective in the management of some patients with basal cell nevus syndrome.

Effects of levamisole in Sézary syndrome. Apparent acceleration of disease.

2 patients with Sézary syndrome were treated with levamisole. Both patients had clinical and hematological evidence of acceleration of their disease and ultimately died, although 1 patient had improvement in skin test reactivity. We suggest that levamisole be used with caution, if at all, in patients with T-lymphocyte malignancies.

Acanthosis nigricans presenting as hyperkeratosis of the palms and soles.

A patient with an unusual form of palmoplantar hyperkeratosis is described. On thorough physical examination, widespread acanthosis nigricans was discovered. We believe this patient's distinctive keratoderma represents acanthosis nigricans.

Disseminated superficial actinic porokeratosis: appearance associated with photochemotherapy for psoriasis.

A patient with chronic psoriasis treated with psoralens plus ultraviolet A (PUVA) developed characteristic lesions of disseminated superficial actinic porokeratosis (DSAP). Since other processes associated with ultraviolet irradiation, including epidermal dysplasia, actinic keratoses, squamous cell carcinomas, and keratoacanthomas, have been reported to result from PUVA, it is possible that her DSAP lesions were induced by this therapy.

Keratitis, ichthyosis, and deafness (KID) syndrome: management with chronic oral ketoconazole therapy.

Extensive cutaneous mycoses have been described in patients with the keratitis, ichthyosis, and deafness (KID) syndrome. We present a case occurring in a 48-year-old woman where improvement in the ichthyosiform dermatosis, stabilization of her ocular disease, and apparent partial prevention of further cutaneous malignancies occurred in association with ketoconazole therapy.

Prenatal diagnosis for keratin mutations to exclude transmission of epidermolytic hyperkeratosis.

Epidermolytic hyperkeratosis (bullous congenital ichthyosiform erythroderma) is an autosomal dominant skin disorder caused by mutations in keratins 1 and 10. We have used direct gene sequencing to ascertain the status of a 15 week fetus of parents whose first child was affected with this disorder. The parents show no clinical signs of epidermolytic hyperkeratosis but were concerned about the possibility of transmitting the disorder due to germline mosaicism. Molecular analysis of the affected son revealed a G to A mutation in codon 156 of keratin 10, resulting in an arginine to histidine substitution within the highly conserved 1A region. Codon 156 has been previously identified as a mutational hot spot and substitutions of this arginine residue are very common in epidermolytic hyperkeratosis patients. Analysis of genomic DNA isolated from amniotic cells showed that the fetus did not harbour this mutation and a healthy infant was eventually born that was unaffected by this disorder.