Multifocal granular cell tumors in a 10-year-old boy.

Multifocal granular cell tumor is a poorly understood entity. Its importance lies in its association with several genetic disorders and the challenging distinction between multifocal and metastatic granular cell tumor. Herein, we report an unusual case of nonmalignant, multifocal, granular cell tumors affecting the skin, including the scrotum, in a 10-year-old boy.

Topical carbocysteine: A new option for the treatment of ichthyosis.

N-acetylcysteine in combination with urea is effective for the treatment of congenital ichthyosis. Although it is well tolerated, its foul smell may compromise treatment adherence. Carbocysteine is a similar molecule without that bad odor. Thus, we have tried a new formula with carbocysteine for the treatment of 4 patients with ichthyosis, with positive results.

Considerations on the programmed functional life (one generation) of a green artificial reef in terms of the sustainability of the modified ecosystem.

The installation of artificial reefs serves to enhance marine ecosystems, although it also modifies them. These changes do not have to be irreversible, since it is possible to treat the functional life of an artificial reef (AR) as a variable factor to be determined, with the objective of contributing to the sustainability of the ecosystem. The quest for sustainability does not end with the manufacture and installation of the AR units. It is also necessary to analyse the sustainability of the modified ecosystem, through the production of services. This leads to consider the medium-term return of the ecosystem to its initial state, once the functional life of the ARs expires. This paper presents and justifies an AR design/composition for limited functional life. It is the result of acting on the base material, the concrete, with the objective of limiting the useful life to one social generation. Four different dosages were proposed for such a purpose. They were subjected to mechanical tests (compressive strength and absorption after immersion), including an innovative abrasion-resistant one. The results allow estimating the functional life of the four types of concrete from the design variables (density, compactness, and quantity of water and cement as well as its relation). To this end linear regression models and clustering techniques were applied. The described procedure leads to an AR design for limited functional life.

Subungual squamous cell carcinoma: a case series.

Subungual tumors are rare in general. Of all tumors, subungual squamous cell carcinoma (SSCC) is the most frequent one. Protean clinical presentations and the lack of awareness of the disease are responsible for an incorrect or delayed diagnosis and subsequent delayed treatment. We have reported here four patients with SSCC who were previously wrongly diagnosed with a benign process and treated unsuccessfully for years. We would like to highlight the need of a biopsy in chronic or recurrent nail lesions that fail to respond to a previous conservative treatment in order to rule out SSCC.

Progressive nodular histiocytoma associated with thrombocytopenia with absent radii (TAR syndrome) and angiofibromas.

A 50-year-old woman was admitted due to a long-standing history of cutaneous lesions, which were gradually increasing in number and size, located on the trunk and extremities. Histological studies confirmed the initial clinical diagnosis of histiocytomas. Moreover, the patient had numerous smooth erythematous papules on her chin and around her nose, which were diagnosed histologically as angiofibromas. The patient had congenital phocomelia. Analytical and imaging studies revealed the presence of bilateral phocomelia due to absent radii and thrombocytopenia (TAR syndrome). Multiple histiocytomas in a normolipaemic patient bring up several differential diagnoses. Slow progressive evolution without spontaneous resolution and a scattered distribution on the trunk and extremities suggest the diagnosis of progressive nodular histiocytoma. To our knowledge progressive nodular histiocytoma has not been reported previously associated either with TAR syndrome or with angiofibromas. These entities are uncommon, thus their association may not be due to chance.

[De Sanctis-Cacchione syndrome]. / Síndrome de De Sanctis-Cacchione.

We present a male patient with photosensitivity since the earliest months of his life, and pigmented macules in exposed areas, some showing clinical atypia, which increased in number over time. Molecular biology studies detected an alteration in DNA repair ability, so xeroderma pigmentosum was diagnosed. Shortly after birth, low weight, microcephaly and psychomotor retardation had been observed, but the cause was not established. The patient progressively showed neurological disorders that included perceptive deafness, hyporeflexia and areflexia, as well as choreoathetotic movements. Therefore, we felt that the patient's symptoms fit De Sanctis-Cacchione syndrome.

Pseudoangiosarcomatous squamous cell carcinoma of skin arising adjacent to decubitus ulcers.

Pseudoangiosarcomatous, or pseudovascular, squamous cell carcinoma of skin is an unusual form of acantholytic (adenoid, pseudoglandular) squamous cell carcinoma that mimics the histolopathologic appearance of angiosarcoma. We report a case of pseudoangiosarcomatous squamous cell carcinoma arising adjacent to decubitus ulcers. The histopathologic examination of a wedge biopsy specimen revealed infiltrative cords of neoplastic cells that formed interanastomosing channels imitating angiosarcoma. Immunohistochemical staining was negative for the endothelial markers (CD31, CD34, and factor VIII-related antigen) and positive using cytokeratin antibodies (AE1/AE3 and 34 betaE12). Because of metastatic disease, palliative measures were undertaken and the patient died four months later. To our knowledge, our patient is the first with pseudoangiosarcomatous squamous cell carcinoma of skin developing within decubitus ulcer.

Mejoría de epidermolisis ampollosa simple con tetraciclina: un ejemplo de ensayo clínico de paciente único en dermatología / Improvement of simple bullous epidermolysis with tetracycline: an example of single patient (N-of-1) clinical trial in dermatology

Describimos un caso clínico de epidermolisis ampollosa simple, singular por su inicio en la sexta década y su asociación con síndrome nefrótico y polineuropatíadesmielinizante. Un ensayo clínico de paciente único nos permitió establecer la utilidad del tratamiento con tetraciclina (500 mg/8 h) paradisminuir el número de nuevas ampollas. Este caso ilustra el empleo del ensayo clínico de paciente único en dermatología

We report a case of epidermolysis bullosa simplex, with singular findings such as late appearance (sixth decade), and associated nephrotic syndromeand demyelinating polyneuropathy. A single patient (N-of-1) clinical trial established the usefulness of tetracycline therapy (500 mg/8 h) to diminishthe number of new bullae. This report serves as an example of the use of single patient clinical trials in dermatology

Síndrome de De Sanctis-Cacchione / De Sanctis-Cacchione syndrome

Se presenta el caso de un paciente con fotosensibilidad desde los primeros meses de su vida y con máculas pigmentadas en áreas expuestas, algunas de ellas con atipia clínica, que fueron aumentando en número a largo del tiempo. Los estudios de biología molecular detectaron alteración en la capacidad de reparación del ADN, por lo que se diagnosticó de xeroderma pigmentoso. Al poco tiempo de nacer ya se había observado retraso psicomotor, bajo peso y microcefalia sin que se filiara la causa. Progresivamente fue manifestando alteraciones neurológicas que incluían sordera neurosensorial, hiporreflexia y arreflexia, así como movimientos coreoatetósicos. Por todo ello consideramos que el paciente puede incluirse en el síndrome de De Sanctis-Cacchione

We present a male patient with photosensitivity since the earliest months of his life, and pigmented macules in exposed areas, some showing clinical atypia, which increased in number over time. Molecular biology studies detected an alteration in DNA repair ability, so xeroderma pigmentosum was diagnosed. Shortly after birth, low weight, microcephaly and psychomotor retardation had been observed, but the cause was not established. The patient progressively showed neurological disorders that included perceptive deafness, hyporeflexia and areflexia, as well as choreoathetotic movements. Therefore, we felt that the patient's symptoms fit De Sanctis-Cacchione syndrome

Asociación de lesiones cutáneas y óseas / Association of skin and bone lesions

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Enfermedad de Hansen: a propósito de dos casos de lepra lepromatosa en Pontevedra / Hansen's disease: two cases of lepromatous leprosy in Pontevedra, Spain

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