We report two patients with severe amicrobial pustular dermatosis with immunological abnormalities: a 63-year-old woman with a 30-year-history of discoid lupus erythematosus and sicca syndrome, and a 35-year-old woman with high levels of gamma-globulinemia and positive antinuclear antibodies. Both patients presented with crusty and eroded erythematous plaques studded with aseptic pustules on the back, face, and scalp. Histological examination showed acanthosis, neutrophilic exocytosis to the epidermis, and neutrophilic and lymphocytic infiltration with nuclear dust in the dermis. These patients were diagnosed as having "amicrobial pustulosis associated with autoimmune diseases". The eruptions improved with combination treatment of oral prednisolone with cyclosporin A or diaminodiphenylsulphone. Although the pathogenesis remains unclear, amicrobial pustular dermatosis might be one of the cutaneous complications in autoimmune diseases.

No abstract

A 68-year-old woman had a peculiar clinical course of cutaneous lymphoma. She first developed nonpuritic erythematous macules with fine scales followed by development of erythematous infiltrated plaques. The clinical course could be interpreted as that of mycosis fungoides. Histologically, the lesions showed pagetoid infiltration of atypical lymphoid cells. Suddenly, with high fever, numerous purpuric, ulcerated, or crusted plaques with underlying infiltration or nodules began to appear on most areas of the patient's body. Biopsy specimens of the lesions revealed angiocentric and angiodestructive infiltration by neoplastic T cells and marked epidermotropism of these cells. These atypical cells immunohistochemically had CD8+ surface phenotype. The patient died of respiratory insufficiency due to acute pulmonary infiltration. Autopsy demonstrated angiocentric and angiodestructive lymphomatous infiltration in the lung.

Double-stranded RNA-activated protein kinase (PKR) is a interferon-induced protein initially known for its inhibitory effects on cellular and viral protein synthesis. In recent studies, PKR has been shown to be an important participant in a broad array of cellular processes, including signal transduction, differentiation, apoptosis, cell growth, and tumorigenesis. The expression of PKR in normal human keratinocytes (NHEK) was examined, and its expression in several skin lesions was compared immunohistochemically with that of proliferating cell nuclear antigen (PCNA). Expression of PKR mRNA was detected in NHEK without IFNgamma treatment; the level of PKR mRNA increased with IFNgamma treatment for two hours. Immunoblot analysis revealed that the monoclonal anti-PKR antibody reacted specifically with a 68kDa PKR protein in extracts from NHEK. Immunohistochemistry revealed that PKR protein was expressed in normal epidermis and mucosa. PKR expression was not restricted only to suprabasal cells but was also observed in basal cells positive for PCNA. In psoriatic plaques, PKR expression was lower in basal and parabasal keratinocytes and comparable in suprabasal keratinocytes to the levels in normal skin. PKR was partially detected in atypical cells in non-invasive keratinocytic neoplasia but was completely absent from undifferentiated tumor cells of squamous cell carcinoma. The present study demonstrated that PKR protein is constitutively expressed in epidermal and epithelial keratinocytes of normal skin and mucosa and indicated that a loss of PKR is not associated with the malignant transformation itself but with the increased cell proliferative activity and the altered differentiation of keratinocytes.

No abstract