We report phakomatosis pigmentovascularis detected in a Caucasian child characterized by the presence of a nevus flammeus and nevus anemicus on the face, a telangiectatic linear nevus of the right leg, and a very extensive blue spot covering 60% of the body surface, with ocular melanosis. Multiple angiomatous lesions of the kidney are associated without alterations of the central nervous system (CNS). This association has not been reported before; it could be a further expression of the complex of developmental defects. Our case corresponds exactly to type IIb in the classification of phakomatosis pigmentovascularis proposed by Hasegawa. As this classification seems very extensive, the higher incidence of cases corresponding to the second subtype suggests that we should identify it by the term phakomatosis pigmentovascularis, while the others could be considered as only very uncommon variants.

Facial Neoplasms↗

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Effects of phlebotomy on urinary porphyrin pattern and liver histology in patients with porphyria cutanea tarda.

Urinary porphyrin profiles and liver histology have been investigated in a group of adult alcoholics with porphyria cutanea tarda (PCT) before and after one year phlebotomy. Both parameters were evaluated during the same period in a group of patients who did not undergo specific therapy for PCT. All patients were advised to abstain from alcohol. At the end of the one year observation period there was a significant fall of urinary total porphyrins and in the uro/coproporphyrin ratio in treated patients compared to basal values whereas no changes were found in controls. Liver biopsy findings revealed a significant reduction of hepatic fatty degeneration and siderosis with no changes in inflammatory infiltrates and fibrosis in treated patients, so the progression of liver disease was similar to controls. These results show that clinical and biochemical remission of PCT can occur independently of the evolution of the concomitant liver disease.

Alcoholism↗

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A case of hypopigmented mycosis fungoides in a young Caucasian boy.

Hypopigmented mycosis fungoides is a variant of mycosis fungoides characterized by the presence of hypopigmented patches as the sole manifestation of the disease. It has been described almost always in young black or dark-skinned patients. The only white patient described was a 64-year-old woman who not only had hypopigmented lesions, but also nodular lesions with lymphadenopathy. We describe hypopigmented lesions arising in a white boy 12 years of age, born in northern Italy, without any foreign ancestors. The microscopic alterations, with epidermotropism, the immunologic markers, the negativity of T-cell receptor gene rearrangement, and the good response to PUVA therapy correspond to the main findings in black patients with this disease. Long-term follow-up of these patients is important to obtain better knowledge of the natural history of the disorder. Hypopigmented mycosis fungoides must now be included in the differential diagnosis of hypopigmented macular lesions not only in black or dark-skinned patients but also in white patients.

Antigens, CD↗

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A feasibility study using polychemotherapy (cisplatin + vindesine + dacarbazine) plus interferon-alpha or monochemotherapy with dacarbazine plus interferon-alpha in metastatic melanoma.

AIMS AND BACKGROUND: This trial evaluated the feasibility and tolerability of an immunochemotherapeutic approach that uses cisplatin, vindesine, and dacarbazine (DTIC), or only DTIC, in combination with interferon alpha-2a (IFN-alpha), in patients with metastatic melanoma, considering the significant toxicity of several different regimens used up to now. METHODS: Between May 1995 and September 1997, 51 melanoma patients (50 of whom were assessable) entered a multicentric trial and were randomized to receive cisplatin (30 mg/m2 daily for 3 days) + vindesine (2.5 mg/m2 only day 1) + DTIC (250 mg/m2 daily for 3 consecutive days) + IFN-alpha (3 MIU i.m. 3x/wk continuously) (CVD arm) versus DTIC (800 mg/m2 day 1) + IFN-alpha (3 MIU i.m. 3x/wk continuously) (DTIC arm). The chemotherapy was recycled every 21 days. Patient reevaluation was performed every two cycles, and the treatment was continued in case of objective response or stabilization of disease. RESULTS: We observed 3 complete responses, 2 partial responses and 5 stable diseases in the CVD arm, and 2 partial responses and 4 stabilizations of disease in the DTIC arm. CONCLUSIONS: We conclude that these chemotherapeutic regimens are well tolerated regimens with modest toxicity. Future trials will be conducted associating the CVD regimen with biological response modifiers (IFN, IL-2) in order to improve the results.

Adult↗

PubMedDate not supplied

Accuracy of epiluminescence microscopy among practical dermatologists: a study from the Emilia-Romagna region of Italy.

AIMS AND BACKGROUND: The accuracy of epiluminescence microscopy (ELM) for pigmented skin lesions (PSLs) is still unclear. The large scale utilization of the technique is generally discouraged. The present study was aimed at comparing the accuracy of ELM with that of clinical examination alone in a group of 20 practical dermatologists. METHODS AND STUDY DESIGN: Thirty digital clinical images of benign and malignant PSLs and their digital ELM counterparts were used. A set of cumulative accuracy measures reported in the combined clinical/ELM diagnosis was compared with that reported in the clinical diagnosis alone. RESULTS: The proportion of nonmelanocytic lesions (NMLs), melanocytic nevi, and melanomas correctly identified, the predictive value of such diagnoses, and the proportion of melanomas detected by referral for biopsy (irrespective of the diagnosis reported) increased significantly. The cases referred for biopsy despite a "benign" impression decreased significantly among NMLs. CONCLUSIONS: The observed tendency towards a greater accuracy suggests that even in the routine dermatology practice ELM has the potential to improve the clinical examination of PSLs.

Dermatology↗

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[Pigmentation abnormalities in the course of topical immunotherapy of alopecia areata].

Pigmentation troubles have been associated in the past years with contact dermatitis and patch-testing. Contact allergy and increase of pigmentation has been associated with Tinopal; on the other hand contact allergy and depigmentation have been associated with many substances such as DNCB, squaric acid dibutylester (SADBE), carbyne, alstroemeria. Leukoderma can also be produced by irritant compound such as phenols, catechols and mercaptoamines. During 1978 and 1984 we have treated 132 patients suffering from alopecia areata with DNCB or SADBE: 51 patients have been treated by DNCB and 81 by SADBE. During the treatment 10 patients developed a leukoderma vitiligo-like localized to the areas of topical application of the allergens and to the flare-up site; one patient had an increase of pigmentation. Lesions vitiligo-like appeared 10-15 weeks after the onset of treatment. Among the patients who developed leukoderma, 4 had a personal history of vitiligo. The possible pathogenetic mechanisms are discussed.

Administration, Topical↗

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