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A 16 year-old boy with haemophilia and acquired immunodeficiency syndrome (AIDS) developed disseminated cryptococcosis and facial skin papules, some of them umbilicated, that showed numerous encapsulated budding yeast organisms suggestive of Cryptocaccus neoformans on cutaneous cytology. Histologic examination confirmed the diagnosis of cutaneous cryptococcosis. to our knowledge, this case is the first cutaneous cryptococcosis in a haemophiliac with AIDS reported in Europe, and the second one resembling molluscum contagiosurn. [ABSTRACT FROM AUTHOR]

Subcutaneous fat necrosis associated with pancreatitis has been postulated to be due to the effects of circulating pancreatic lipase. We report positive intracellular staining of adipocytes with a monoclonal antibody to pancreatic lipase in a lesion of subcutaneous pancreatic fat necrosis, and confirm the role of pancreatic lipase in the pathogenesis of this syndrome. [ABSTRACT FROM AUTHOR]

A patient with keratoderma palmoplantaris striata is presented. In the family tree there are 36 relatives affected to a varying degree. The familial incidence is the largest reported in the literature reviewed. The response to etretinate was excellent, controlling the lesions with minimal side-effects. [ABSTRACT FROM AUTHOR]

The human hematopoietic progenitor cell antigen (CD34) is a cell surface protein expressed by human hematopoietic progenitor cells, vascular endothelium, and many mesenchymal tumors. Sections from six samples of normal skin and from 41 epithelial tumors of the skin were studied. Immunostaining of epithelial cells from the external root sheath below the attachment of the arrector pili muscle and above the matrix cells was noted in normal samples. Tumors derived from or differentiated toward cells of the outer sheath, especially trichilemmomas, were immunostained with QBEND/10 (anti-CD34 antibody), whereas other epithelial tumors studied were negative. CD34 could serve as a marker of outer sheath cell derivation and may well be of value in the distinction between trichilemmomas and other lesions with similar histopathological features.

Porphyria cutanea tarda (PCT), a relatively uncommon disease, has recently been reported in patients infected with the human immunodeficiency virus (HIV). Although PCT and HIV infection may co-exist by chance, the increasing number of reported cases suggest that HIV or an associated factor triggers the development of PCT in predisposed individuals. We report four additional cases of PCT in HIV seropositive patients and review the previously reported cases. The possible links between PCT and HIV are discussed. We believe the diagnosis of PCT should prompt investigation for HIV infection in all patients.

The pruritic papular eruption of the acquired immunodeficiency syndrome is characterized by generalized, pruritic, skin-colored papules and nodules. Chronic lesions are excoriated and hyperpigmented. The eruption and pruritus typically wax and wane and are resistant to oral antihistamine and topical steroid therapy. The characteristic histologic features are (1) superficial and mid dermal perivascular and perifollicular mononuclear cell infiltrate with numerous eosinophils and (2) follicular damage of varying degrees. When compared with control subjects, these patients did not demonstrate any significant difference in laboratory or demographic data.

A case of delayed onset (17 years) progressive symmetric erythrokeratodermia is presented. The treatment with etretinate initially improved the clinical picture, but it had to be discontinued because of personality changes, particularly depressive crisis. In addition, clinical manifestations, histology, kinetics and ultrastructural changes in this condition are also commented on.

We report a case of systemic mastocytosis in a 21-year-old male with skin, liver and bone marrow involvement. During the clinical course he developed important gastrointestinal manifestations including a peptic ulcer probably caused by mast cell mediators. In this article we also review the most remarkable characteristics of mastocytosis, especially their systemic forms.

After a brief introduction about the classification and clinicopathological findings of acanthosis nigricans, we present a clinical case of the familial type of acanthosis nigricans in a woman with lesions beginning in childhood and with three family members affected. We comment the characteristics that permit us to individualize this condition from the other forms of benign acanthosis nigricans.