Two cases of granulomatous dermatitis with eosinophilla (Wells' syndrome) are reported. With Wells' original four cases, these two cases define a distinctive dermatosis with onset as cellulitis and formation of solid edema and either final spontaneous resolution or resolution with steroid therapy. Microscopic study showed diffuse tissue eosinophilia and fibrinoid flame figures, evolution of associated focal necrobiosis, and formation of focal microgranulomas associated with eosinophils. Biopsy of muscle and fascia showed comparable fasciitis and eosinophilic myositis. Immunofluorescence in one case disclosed fibrin in the dermis and lgM, lgA, and C3 in the blood vessels of the muscle. Recurrences of the lesions often appeared to be related to drug administration or surgery.
Four patients with recessive dystrophic epidermolysis bullosa and 17 invasive primary cutaneous squamous cell carcinomas (SCCs) are presented. All skin cancers arose at the site of scarring due to recessive dystrophic epidermolysis bullosa. Three of 17 (18%) primary cutaneous SCCs recurred locally following initial treatment with either surgical excision or wide surgical excision. Patients with recessive dystrophic epidermolysis bullosa are more likely to develop SCCs of the extremities than nonimmunocompromised patients or patients with epidermodysplasia verruciformis. Like patients with epidermodysplasia verruciformis, patients with recessive dystrophic epidermolysis bullosa are likely to suffer from invasive and metastatic SCCs at a much younger age than nonimmunocompromised patients.
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