PUVA therapy in persistent cutaneous histiocytosis: Case report and literature review.

Background: Cutaneous and mucocutaneous histiocytosis (group C) comprise a wide variety of entities affecting skin and/or mucosae. Although they are considered as reactive proliferations, their exact pathophysiology remains unknown and, therefore, they lack a specific treatment.

Aims: The aim of this study is to review the evidence on cases of histiocytosis treated with UVB and/or UVA and to report a new case of relapsing group C histiocytosis that has been successfully treated with PUVA therapy.

An immunohistochemical study of verrucous hemangiomas.

Background: According to the International Society for the study of vascular Anomalies, vascular anomalies are classified as vascular neoplasms and vascular malformations. In some vascular lesions, categorization as a neoplasm or malformation has not been established with confidence so far. In order to further clarify the nosology of verrucous hemangioma, we studied 13 cases.

Leydig cell tumour: enucleation as a therapeutic choice in a case with atypical symptoms.

Objective: Testicular tumours are rare in paediatric patients, accounting for only 1% of tumours in boys. Leydig cell tumours are the most common gonadal stromal tumours. Since these tumours are functionally active, secreting testosterone, they characteristically produce isosexual pseudoprecocious puberty (IPP), the customary therapeutic choice being radical orchiectomy.

Leukonychia totalis associated with multiple pilar cysts: report of a five-generation family: FLOTCH syndrome?

The association of familial totalis leukonychia with multiple pilar cysts is a rare condition that could represent a separate syndromic entity. Since Bauer described a family with totalis leukonychia and sebaceous cysts in 1920, only four new affected families have been reported. We report a five-generation family with a total leukonychia and multiple pilar cysts on the scalp.

Capillary-lymphatic malformation, kaposiform hemangioendothelioma and delayed Kasabach-Merritt phenomenon.

According to International Society for the Study of Vascular Anomalies classification, vascular anomalies are mainly divided into two groups: vascular tumors and vascular malformations. Nevertheless, a small group of patients present clinical and/or histopathologic overlapping features. We report a case of a 4-month-old boy that presented a vascular lesion on his right buttock with involvement of abdominal wall muscles, abdominal cavity and drainage to primitive iliac by a tortuous drainage vein.

Necrotizing vasculitis with a polyarteritis nodosa-like pattern and selective immunoglobulin A deficiency: case report and review of the literature.

Selective immunoglobulin A deficiency (IgAD) is a primary immunodeficiency disease characterized by low levels (< 7 mg/dl) of serum immunoglobulin (Ig) A and normal serum levels of IgG and IgM. Patients with IgAD have increased risk for recurrent respiratory and gastrointestinal infections, autoimmune disease, asthma and allergy. A 26-year-old woman was admitted with sudden onset of painful cutaneous lesions on her lower extremities, pyrexia and arthromyalgia.

Three cases of cutaneous phaeohyphomycosis by Exophiala jeanselmei.

Cutaneous phaeohyphomycosis is a rare opportunistic fungal disease, knowledge of which is important because of the increase in organ transplantation, aggressive treatments for malignancies, and chronic use of corticosteroids. We report 3 cases of cutaneous phaeohyphomycosis: two patients treated with oral corticosteroids and one elderly woman with multiple hospitalizations. They showed several different clinical appearances.

Papular elastorrhexis, a distinctive entity?

Papular elastorrhexis is a rare entity of elastic tissue characterized by multiple white papules usually located on the trunk. We report a case of papular elastorrhexis in a 22-year-old man with sparse lesions and discuss the main controversial facts in this entity: its consideration as an independent disease or as an incomplete form of Buschke-Ollendorff syndrome and the confusion with and its possible inclusion as a variant of elastic tissue naevi.

Neutrophilic dermatosis of the hands: presentation of eight cases and review of the literature.

Pustular dermatosis of the dorsal hands is a peculiar variant of Sweet's syndrome described by Strutton et al. Nevertheless, not all authors accept this entity. We present a retrospective study of eight cases of neutrophilic vasculitis of the hands.