Successful treatment of generalized elastolytic giant cell granuloma with psoralen-ultraviolet A.

Elastolytic giant cell granuloma (EGCG) is an infrequent granulomatous skin disorder with variable response to different therapeutic regimens. Information on the benefit of phototherapy is very scarce as this therapy has seldom been tried in the affected patients. We present the results achieved in two female patients after undergoing psoralen-ultraviolet A (PUVA). Two 54-year-old otherwise healthy female patients received a course of PUVA after trying other alternatives. Complete clearance was achieved in the two patients with excellent tolerance and no adverse effects. We consider PUVA is a well-tolerated, safe, and effective treatment for patients with EGCG.

Multiple congenital familial smooth muscle hamartoma in two siblings.

Smooth muscle hamartoma (SMH) is a cutaneous malformation mainly composed of a disorganized proliferation of normal muscle fibers that arise from arrector pili. It usually presents as a single congenital lesion that frequently involves the back and the lower limbs. Unusual clinical presentations, such as atypical localizations, multiple disseminated lesions, and generalized forms have been rarely described. In 2001, Gualandri et al. reported the presence of multiple SMH in three members of the same family, namely two brothers and their mother. This is, as far as we know, the only familial case reported in the English literature. We herein describe a similar case affecting two siblings who presented with identical congenital lesions in the same location.

Coexistence of two types of clinical lesions in childhood-onset mastocytosis.

The vast majority of mastocytosis appear in childhood, urticaria pigmentosa (UP) and mastocytomas being the most common types. Terms such as "xanthelasmoid mastocytosis", "pseudoxanthomatous mastocytosis" or "nodular mastocytosis" have been introduced in the literature to describe the presence of yellowish papular or nodular lesions. We describe two children with cutaneous mastocytosis showing yellowish lesions in combination with other skin lesions. A 10-year-old girl presented with asymptomatic lesions in her vulva at birth, and developed brownish macules on her trunk years after. An eight-year-old boy presented with multiple yellowish papular lesions on his trunk, neck and limbs coexisting with a few clinically anetodermic lesions. No systemic involvement was found and the skin biopsy confirmed a cutaneous mastocytosis in both cases. The two patients are currently asymptomatic and are being periodically followed up. Mastocytoses may show a variety of clinical lesions, sometimes leading to misdiagnosis. Although there are previous reports, involvement of the mucosae and secondary anetoderma are not common findings in cutaneous mastocytoses. We consider that cutaneous manifestations of mastocytoses compose a clinical spectrum, thus explaining the coexistence of different clinical lesions and the development of uncommon presentations.

Perforating neutrophilic and granulomatous dermatitis of the newborn--a clue to immunodeficiency.

We report two newborns with a widespread cutaneous eruption consisting of discrete papules which evolved into vesicles, pustules, crusts, and ulcers. These healed over a 2-week period with scarring. Histopathology showed three main features--histiocytic granulomas, neutrophilic infiltration, and transepidermal elimination of degenerated collagen and debris through hair follicles. Both patients had congenital immunodeficiency. This skin condition of the newborn, with distinct clinical and histopathologic features, is a manifestation of immunodeficiency that has not been previously described.

Molecular analysis in two beta-mannosidosis patients: description of a new adult case.

beta-Mannosidosis is a lysosomal storage disorder caused by deficiency of beta-mannosidase. Thirteen families with cases of beta-mannosidosis have been described including one case previously reported by our group. We present clinical and biochemical data in a new adult case, and the molecular analyses in both this new case and the one previously reported. We detected four novel mutations: p.R182W, p.G392E, p.W466X and c.1848delA. Discrepancies between genomic DNA and cDNA results when detecting this last deletion suggested a nonsense-mediated decay cell process (NMD).

Multinucleate cell angiohistiocytoma. Report of five cases.

BACKGROUND: Multinucleate cell angiohistiocytoma is an infrequent and most likely non-neoplastic disorder usually seen in acral regions in elderly women. It presents clinically as asymptomatic red-to-brown tumors, with a tendency to confluence. It must be distinguished from other diverse cutaneous lesions, notably dermatofibroma, Kaposi sarcoma, and angiofibroma. METHODS: We report the clinical, histopathological, and immunohistochemical findings of five patients, all women aged between 51 and 78 years. All except the first presented lesions on both of the lower limbs. None of the patients developed spontaneous resolution of the lesions and one was successfully treated by cryosurgery. COMMENTS: Multinucleate cells are characteristic, but neither exclusive nor pathognomonic, of multinucleate cell angiohistiocytoma, since they can also appear in other inflammatory, neoplastic, or reactive processes. The presence of these cells and vascular proliferation in dermis media are the principal histopathological findings in this infrequent entity. In immunohistochemical studies, the multinucleate cells are often positive for vimentin and factor XIIIa.

Día del Euromelanoma. Resultados en España de las campañas de 2000, 2001 y 2002 / Euromelanoma day. Results of the 2000, 2001 and 2002 campaigns in Spain

Fundamento. La Academia Europea de Dermatología instauró en el año 2000 una campaña de detección precoz de melanoma denominada «Día del Euromelanoma». Objetivo. Exponer los resultados españoles del «Día del Euromelanoma », en las campañas realizadas en los años 2000, 2001 y 2002. Material y métodos. Los miembros de la Academia Española de Dermatología participantes realizaron una revisión gratuita de cuantas personas pidieran cita para la detección de lesiones sospechosas. A los 4 meses de la visita, se localizó telefónicamente a todos los pacientes que habían sido diagnosticado de sospecha de melanoma para preguntar si las lesiones habían sido extirpadas y, en su caso, el resultado del estudio histológico de la pieza. Resultados. Se recibieron 33.750 llamadas para concertar cita y fueron examinados 12.487 pacientes. La participación media de dermatólogos fue de 399 académicos por año. Se detectaron un total de 164 lesiones sospechosas de melanoma, sobre todo en Andalucía y Cataluña, confirmándose histológicamente en 31 casos. La correlación clinicopatológica fue del 23,3 %, cifra mayor que la de Estados Unidos y similar a la de países mediterráneos. El espesor tumoral medio fue de 0,93 mm. Conclusiones. Aunque estas campañas tienen los inconvenientes de posibles sesgos en sus hallazgos y producir cierta alarma social, el beneficio para la población ha sido incuestionable en términos de concienciación hacia un problema sanitario

Background. In the year 2000, the European Academy of Dermatology and Venereology initiated a campaign, called «Euromelanoma Day,» for the early detection of melanoma. Objective. To discuss the results of «Euromelanoma Day» in Spain for the campaigns carried out in 2000, 2001 and 2002. Material and methods. Participating members of the Spanish Academy of Dermatology examined everyone who requested an appointment, free of charge, in order to detect suspicious lesions. Four months after the visit, telephone contact was made with all patients who had been diagnosed with suspected melanoma to ask them whether the lesions had been excised, and if so, the result of the histological study of the specimen. Results. 33,750 calls to set up an appointment were received, and 12,487 patients were examined. The average participation by dermatologists was 399 academy members per year. A total of 164 lesions suspected of being melanoma were detected, above all in Andalusia and Catalonia, and the diagnosis was histologically confirmed in 31 cases. The clinicopathological correlation was 23.3 %, a higher figure than the one for the USA and similar to the one for Mediterranean countries. The average thickness of the tumors was 0.93 mm. Conclusions. Although these campaigns have the drawback of possible bias in the findings and may cause some degree of public alarm, the benefit to the population was unquestionable in terms of heightening awareness of a health problem

[Euromelanoma Day. Results of the 2000, 2001 and 2002 campaigns in Spain]. / Día del Euromelanoma. Resultados en España de las campañas de 2000, 2001 y 2002.

BACKGROUND: In the year 2000, the European Academy of Dermatology and Venereology initiated a campaign, called "Euromelanoma Day," for the early detection of melanoma. OBJECTIVE: To discuss the results of "Euromelanoma Day" in Spain for the campaigns carried out in 2000, 2001 and 2002. MATERIAL AND METHODS: Participating members of the Spanish Academy of Dermatology examined everyone who requested an appointment, free of charge, in order to detect suspicious lesions. Four months after the visit, telephone contact was made with all patients who had been diagnosed with suspected melanoma to ask them whether the lesions had been excised, and if so, the result of the histological study of the specimen. RESULTS: 33,750 calls to set up an appointment were received, and 12,487 patients were examined. The average participation by dermatologists was 399 academy members per year. A total of 164 lesions suspected of being melanoma were detected, above all in Andalusia and Catalonia, and the diagnosis was histologically confirmed in 31 cases. The clinicopathological correlation was 23.3 %, a higher figure than the one for the USA and similar to the one for Mediterranean countries. The average thickness of the tumors was 0.93 mm. CONCLUSIONS: Although these campaigns have the drawback of possible bias in the findings and may cause some degree of public alarm, the benefit to the population was unquestionable in terms of heightening awareness of a health problem.