A case of pyoderma gangrenosum is described in a girl aged 4. The condition was associated with selective IgA deficiency. The father and the 2 brothers suffered from the same deficiency (autosomal dominant transmission). Treatment with prednisolone and clofazimine produced an excellent clinical response.
A case of pigmented hidroacanthoma simplex showing malignant transformation into porocarcinoma is reported. Although no intracellular duct formation could be observed as in benign tumors, ultrastructurally the tumor cells showed characteristics similar to those of eccrine poroma. Many melanocytes were seen dispersed within the tumoral nests. The melanocyte-keratinocyte relationship was found similar to that occurring in melanoacanthoma. Porocarcinoma cells showed ultrastructural features similar to those of benign cells.
A family is described in which two sisters and their two female fifth-cousins were cases of mandibuloacral dysplasia. This syndrome is characterized by mandibular hypoplasia, delayed cranial suture closure, dysplastic clavicles, abbreviated terminal club-shaped phalanges associated with acroosteolysis and atrophy of the skin over hands and feet. Furthermore, in this family alopecia and loss of the lower teeth were noted. We stress the importance of the cutaneous alterations and the possible existence of partial froms of the disease as in one of the patients presented.
The clinical course of a patient with Sézary syndrome presenting under bullous form is described. The bullae were flaccid on a non-erythematous base. Indirect and direct immunofluorescence were both negative. In the peripheral blood WBC was high (54,000/mm3) and large cells with T lymphocyte properties and small cells with ‘null’ cell features were present. Blister fluid contained only large, E-rosette-positive and α-naphthylacetate esterase-positive Sézary cells. A remarkable spontaneous cyclicity was observed: marked reduction in the number of large circulating Sézary cells each time bullae appeared. This suggests that only the large cells were capable of migrating to the skin and from the skin to peripheral blood.
The case is reported of a 30-year-old man suffering from dyskeratosis congenita with severe pancytopenia, in association with generalized verrucosis. Viral particles were detected electron microscopically. The importance of an impaired immunological state, demonstrated in this case by lymphocyte stimulation and rosette tests, is discussed.
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