Scleromyxedema: successful treatment of cutaneous and neurologic symptoms.

Scleromyxedema is a rare systemic disorder characterized by cutaneous sclerosis and papulosis, accompanied by deposition of mucin in the skin and other organs. We describe a case of scleromyxedema in a 62-year-old man. The cutaneous symptoms of the disorder were preceded by episodes of acute central nervous system dysfunction that included mental confusion, hemiparesis, tremor, and migraine. As the cutaneous symptoms progressed, the patient experienced persistent confusion and difficulty concentrating. Therapy with melphalan and plasmapheresis led to complete resolution of the cutaneous symptoms as well as near-resolution of the neurologic symptoms. This is the first report to describe the successful treatment of the cutaneous symptoms of scleromyxedema accompanied by reversal of chronic neurologic dysfunction.

Ulcerated necrobiosis lipoidica diabeticorum in a patient with a history of generalized granuloma annulare.

Granuloma annulare and necrobiosis lipoidica diabeticorum have rarely been reported in the same patient. We describe the unusual case of a woman with diabetes and a history of generalized granuloma annulare who noted leg ulcers that clinically represented ulcerated necrobiosis lipoidica diabeticorum and had histologic features of necrobiosis lipoidica diabeticorum and granuloma annulare. Her condition responded to treatment with antiplatelet agents.

The role of lymph node dissection for clinical stage I malignant melanoma of intermediate thickness (1.51-3.99 mm).

The survival times of patients who had an elective regional lymph node dissection was compared with that of those who did not undergo the procedure in a database of 72 patients with clinical Stage I melanoma of intermediate thickness (1.51-3.99 mm). All of the patients had been followed for 5 years or longer or until death. No significant differences were found in other reported prognostic factors, suggesting that the two groups were comparable. By multivariate analysis, a low mitotic rate, intermediate patient age, and the presence of an infiltrative lymphocytic response were found to be associated with favorable survival. There did not appear to be any association of elective regional lymph node dissection with survival; and it was concluded that such therapy should not be regarded as "standard" for clinical Stage I melanoma of intermediate thickness.

Lack of association between intraocular melanoma and cutaneous dysplastic nevi.

The occurrence of uveal and cutaneous malignant melanoma and the dysplastic nevus syndrome in the same individual suggests an etiologic relationship among these diseases. Thus, the dysplastic nevus syndrome could be viewed as marking an increased risk of both cutaneous and ocular melanoma. We postulated that if such a relationship exists, patients with both forms of melanoma should have a high prevalence of dysplastic nevi. We examined 44 patients (31 women and 13 men ranging in age from 20 to 80 years) with uveal melanoma for evidence of cutaneous melanoma and dysplastic nevi. We also examined photographs of 46 patients (24 men and 22 women ranging in age from 19 to 67 years) with nonfamilial cutaneous melanoma to determine the prevalence of dysplastic nevi. We found a 4.5% prevalence of dysplastic nevi in patients with uveal melanoma, significantly lower than the 41% prevalence in patients with cutaneous melanoma (two of 44 patients vs 19 of 46 patients). This study indicates that uveal and cutaneous melanoma are not etiologically linked through dysplastic nevi and suggests that patients with uveal melanoma are no more likely to have cutaneous dysplastic nevi than the general population.

Target blue nevus.

In two cases of target blue nevi of the foot, both lesions displayed a distinctive concentric pigmentary variation. This correlated histologically with a peripheral alteration of dermal collagen accompanied by a focal regression or differentiation of blue nevus cells. The resulting distinctive target blue nevus can be clinically differentiated from a malignant melanoma.

Skin markings in malignant melanoma.

Photographic analysis of the skin markings in 92 malignant melanomas (66 superficial spreading, seven nodular, 12 acral lentiginous, and seven lentigo maligna melanomas) was undertaken. Skin markings are uniformly eradicated in areas of vertical growth phase disease and sometimes in areas of regression. Not only were skin markings always preserved in areas of radical growth phase disease, but they were sometimes accentuated. For all types of melanoma, loss of skin markings is a poor diagnostic criterion because it is a late sign associated with invasion of the dermis and thus a deteriorating prognosis.

Malignant angioendotheliomatosis proliferans treated with doxorubicin.

An 84-year-old man was examined for progressive pain, edema, and infiltrating skin lesions on his lower extremities. A skin biopsy specimen confirmed the suspected diagnosis of angioendotheliomatosis proliferans. The clinical course and histologic changes suggested the malignant form of this disease. Treatment with intravenous doxorubicin hydrochloride caused a complete clinical remission.

Topical chemotherapy of dysplastic melanocytic nevi with 5% fluorouracil.

A therapeutic response to topical fluorouracil in a patient with the large atypical nevus syndrome is presented. Six of the patients' dysplastic melanocytic nevi were treated with topical fluorouracil. Four common acquired melanocytic nevi from normal individuals were also treated. All six dysplastic nevi responded with inflammation, ulceration, and subsequent disappearance of the lesion, while the four control melanocytic nevi remained unchanged. Although we do not presently advocate the therapeutic use of fluorouracil for large atypical nevi until its safety and efficacy have been established, the response reported herein may herald an important advance in the therapy of these troublesome lesions that are known to be histogenetic precursors of malignant melanoma.

Clinical differentiation of adult-onset cavernous angioma from nodular malignant melanoma.

Asymptomatic nodules, suspected of being nodular melanomas, were subjected to 30 s of direct pressure. All the biopsy-proven nodular melanomas remained unchanged by this procedure. The nodules later proven by biopsy to be angiomas were found to collapse slowly in response to pressure. This test can be used to identify angiomas. It should be employed in the routine clinical examination of lesions suspected of being nodular melanomas.