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.

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.

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.

[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.