Familial mastocytosis associated with neurosensory deafness.

Mastocytosis is a disease characterized by excessive accumulation of mast cells in different tissues and symptoms caused by the release of mast cell mediators. The skin is frequently directly involved in mastocytosis. The disease is rarely seen in other members of the subjects' family; only 49 cases of familial mastocytosis have been reported. Familial mastocytosis associated with hearing loss may represent a newly described inherited entity. We describe a brother and sister exhibiting skin mastocytosis and neurosensory deafness, associated with a history of hearing loss in their father's family. The appearance of the mast cell disease in two siblings, who presented with similar clinical features represents a familial form of mastocytosis; the association with an inherited form of deafness may constitute a new syndrome. Our patients show several features similar to some previously reported cases but different insofar that additional congenital defects and mental retardation are absent.

Kindler syndrome: report of a case with ultrastructural study and review of the literature.

Kindler syndrome is characterized by a generalized, progressive poikiloderma with cutaneous atrophy, congenital acral skin blistering, and photosensitivity. Since the first description, approximately 70 cases have been reported worldwide, but ultrastructural studies were performed in only five patients. In none of these patients were biopsies done at birth. In our patient ultrastructural studies were performed both of the blister at birth and of the poikilodermatous and atrophic skin at 6 years of age. Some ultrastructural features in the context of a bullous disease of the newborn that resembles epidermolysis bullosa, should alert investigators to the possibility of Kindler syndrome even in absence of the typical clinical signs.

The nails in psoriatic arthritis.

Nail involvement is often present in patients suffering from psoriatic arthritis. Up to the present, no data concerning the rate of onychopathy associated with psoriatic arthropathy have been reported. 52 patients with psoriatic arthropathy have been studied with particular attention to the possible presence of and the typical features of onychopathy. Nail changes were noted in 86.5% of patients affected by arthropathic psoriasis. The commonest toenail alteration was subungual hyperkeratosis, while the most frequent fingernail change was pitting.

Wilms' tumor with transient dermolysis of the newborn: recurrence of skin lesions during chemotherapy.

Transient bullous dermolysis of the newborn consists of congenital skin defects and a tendency for blistering of the skin and mucous membranes during the neonatal period. A case of transient bullous dermolysis of the newborn associated with Wilms' tumor is reported. Transient bullous dermolysis of the newborn does not represent an obstacle to administering appropriate chemotherapy when simple precautions are taken.

[Tungiasis. A clinical case]. / Tungiasi. Un caso clinico.

Tungiasis is a cutaneous infestation by the female sand flea, Tunga penetrans. Because of the increase in international travel, the disease is reported in Europe, in spite of it being formerly restricted to the equatorial zones. This report describes a case of tungiasis and discusses clinical features, diagnosis and treatment.

Hairy leucoplakia in liver transplant patient.

Oral hairy leucoplakia has been described only in patients infected with the human immunodeficiency virus (HIV) and is a significant predictor for the subsequent development of AIDS. The occurrence of hairy leucoplakia in a liver transplant patient suggests that the lesion is not restricted to HIV seropositive individuals, but can be found in other categories of immunosuppressed patients.

[Congenital transient mechanobullous dermatosis (Bart's syndrome). A clinical case and review of the literature]. / Congenital transient mechanobullous dermatosis (sindrome di Bart). Un caso clinico e review della letteratura.

Bart's syndrome or congenital transient mechano-bullous dermatosis is one of the lesser known presentations of epidermolysis bullosa. It is characterized by congenital skin defects and by a tendency for blistering of the skin and sometimes of mucous membranes and sometimes associated with nail deformities, all of which remitted within in a few years. A neonate is described and compared with similar patterns from the literature.

[Dermatologic problems in patients undergoing liver transplantation]. / Problemi dermatologici in pazienti sottoposti a trapianto di fegato.

Skin troubles were studied in 28 liver transplant patients treated with immunosuppressive agents. Skin complications ranged from minor annoyances to life-threatening conditions. The frequency of dermatologic problems needs periodic examinations by a dermatologist.