Pemphigus vulgaris in association with silicosis.
We report on a sixty-seven year old miner with pemphigus vulgaris characterised clinically by a three month history of relapsing oral lesions and blisters/erosions on the trunk, axillae and extremities, histologically by suprabasal cleavage due to acantholysis, immunologically by the epidermal intercellular net-like pattern due to deposits of IgG- and IgM-antibodies and complement C3 in the direct immunofluorescence as well as by serum antibodies to desmoglein 3 (130 KD) and plakoglobin (85 KD) by immunoblotting analysis. Silicosis has already been known for 6 years. In addition, antinuclear antibodies, anti-ssDNA-antibodies and anti-topoisomerase antibodies were found. Clinical improvement and clearing of skin symptoms could be achieved by systemic steroids in combination with cyclophosphamide. However, the patient died of sepsis deriving from recalcitrant pneumonia. Although the association of silicosis with various autoimmune diseases such as systemic sclerosis, systemic lupus erythematosus, rheumatoid arthritis and dermatomyositis has been reported many times, our patient is, to the best of our knowledge, the second case with features of the two diseases: pemphigus vulgaris and silicosis.
Haustein
U F
UF
University of Leipzig, Department of Dermatology, Liebigstr. 21, D-04103 Leipzig, Germany. ufh@medizin.uni-leipzig.de
D000368:Aged; D003520:Cyclophosphamide / Q000008:administration & dosage; D018450:Disease Progression; D004359:Drug Therapy, Combination; D017809:Fatal Outcome; D006801:Humans; D008297:Male; D008906:Mining; D016273:Occupational Exposure / Q000009:adverse effects*; D010392:Pemphigus / Q000150:complications* / Q000175:diagnosis* / Q000188:drug therapy; D011014:Pneumonia / Q000150:complications / Q000175:diagnosis; D012829:Silicosis / Q000150:complications* / Q000175:diagnosis*; D013256:Steroids / Q000008:administration & dosage
D013256:Steroids; D003520:Cyclophosphamide