We present a 66-year-old man who had maculopapular pigmented lesions on the skin of the head, neck and trunk suggesting generalized eruptive histiocytoma (GEH). These lesions had a yellowish centre in a target-like pattern that has not been previously described. The patient suffered from diplopia and had a severe sensorimotor polyneuropathy causing progressive paresis of the limbs. The explorations performed disclosed the presence of specific xanthomatous infiltrates in the skin, lungs, respiratory tract, peripheral nerves and meninges, suggesting xanthoma disseminatum (XD) or juvenile xanthogranuloma. Multiple osteolytic lesions of large bones were also found. The infiltrate was CD68, MAC 387 and factor XIIIa positive and S-100 and CD1 negative. Some cells contained worm-like bodies visible by electron microscopy. Our patient presented clinical and immunohistochemical findings suggestive of GEH, juvenile xanthogranuloma or XD, supporting the idea of a wide spectrum of non-Langerhans cell histiocytosis. These specific target-like xanthomatous lesions seem to be unique for this new variant of XD.
A 63-year-old woman developed a clinical and pathological picture of polyarteritis nodosa (PAN). Systemic lupus erythematosus (SLE) had been diagnosed 12 years previously, and she had been symptom and therapy-free for a decade. Development of 'primary' systemic vasculitis in SLE patients has rarely been described previously and the significance of this association remains unclear. The possible explanation for this transition from one connective tissue disease to another is discussed.
We report a 61-year-old man with hypermelanosis. marked diffuse lichenification and cutaneous lichen amyloidosus. The amyloidosis was attributed to drug intake.
We report the case of a 45 year-old woman with systemic lupus erythematosus (SLE), who developed clinical and echocardiographic signs of hypertrophic cardiomyopathy. Neither a family history of cardiomyopathy or sudden death nor a personal history of hypertension or valvular lesions were present. The association of SLE with hypertrophic cardiomyopathy has been previously described in only 2 patients.
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