Molluscum pyogenicum: distinctive pyoderma associated with congenital agammaglobulinemia.
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Recurrent gangrenous leg ulcers were associated with a monoclonal IgGk cryoglobulinaemia, though without overt myeloma. Cryoprecipitation of the serum in vitro gave a reversible gel with microtubular structure, built from the monomer and with a high degree of order. The same structure was found distending the superficial vessels of the ulcer biopsy, as well as extravascularly. There was no vasculitis, and luminal obstruction appeared responsible for the ulceration. Microtubular structures occur in nature among diverse proteins and viruses. The protein studied here is similar to a reported instance of myeloma cryo-IgGk.
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Pilonidal sinus is of the same origin as the Pyodermia fistulans sinifica . Both diseases are retention dermatopathies . These sinuses are not of congenital origin. Our observations have made an acquired origin seem more likely. Deep skin folds in the rima ani, the stiffness of the body hairs, the rolling movement of naturally separated hairs push the hair through the skin like a pin. If a hair is rubbed, it moves in the direction of its root with the peripherally directed hair scales. Excision of the sinus area and transplantation of an epithelial flap is the best therapy.
We describe a 14-year-old girl with fatal infectious mononucleosis with high fever, pancytopenia, and multiple skin ulcers due to Staphylococcus aureus. Immunological studies revealed low serum IgM, low natural killer (NK) activity, and high CD4/CD8 ratio. Her father had also low NK activity and high CD4/CD8 ratio. It is suggested that she had a dominantly inherited immunodeficiency predisposing to severe Epstein-Barr virus infection.
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