A 21-year-old woman who had a 2-year history of mixed connective tissue disease (MCTD) developed rapidly evolving ulcers consistent with livedoid vasculitis (LV) in all distal extremities. She presented clinically with Raynaud's phenomenon, polyarthritis and swollen hands; serologically with high titres of ANA and anti-nRNP; and immunogenetically with HLA-DR4 and HLA-DR53. Although there was initial success in treatment except for the skin defects over the ankles, the patient died from disseminated intravascular coagulation. We suggest that LV may be a poor prognostic manifestation in MCTD.

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We report two infants, aged 4 and 9 months, respectively, who had recurrent cutaneous lesions of Langerhans cell histiocytosis confirmed by immunohistochemical and ultrastructural studies. The lesions were confined to the skin and appeared as transient papules on the trunk in both patients. Since neither showed evidence of internal organ involvement or further recurrences of the skin lesions during follow-up periods of 22 and 24 months, respectively, no therapy was given. These cases may be considered as an uncommon benign variant of the Langerhans cell proliferative disorder.