The unusual genetic disorder epidermolysis bullosa dystrophica has been reported in several patients in whom the chronic cutaneous scars led to the development of cutaneous squamous cell carcinoma. However , only one of these previously reported cases involved the autosomal dominant form of the disease; the remainder occurred in its recessive counterpart. We report the second and third patients with squa-mous cell carcinoma associated with the dominant form of epidermolysis bullosa dystrophica. In addition , we not only observed the previous electron microscopic findings of decreased numbers of anchoring fibrils beneath the basal lamina but have also noted marked disruption of the basal lamina itself. Cancer 47:615-620, 1981. HE DOMINANT FORM of epidermolysis bullosa T dystrophica is an uncommon genodermatosis. The basic defect has been demonstrated to occur high in the dermis with blisters occurring beneath the basal lamina. Previous electron microscopic studies have shown the primary abnormality to be a defect in the anchoring fibrils beneath the basal lamina. Our study not only shows abnormalities in the anchoring fibrils but also reveals actual disintegration of the basal lamina itself into what appears to be amorphous debris. In addition, this presentation of a 59-year-old male and his 55-year-old cousin constitutes the second and third reported cases of the dominant form of epidermolysis bullosa dystrophica associated with squamous cell carcinoma in the areas of chronic atrophic scarring. Case Reports Case I A 59-year-old male with a lifelong history of blistering disease of the skin was first seen at the Dermatology Clinic at From the Roswell Park Memorial Institute in 1972. He was referred t'oi evaluation of possible squamous cell carcinomaon his leg. He had a history ofeasy blistering with trauma involving skin surfaces since childhood. The blistering had become more severe on the extremities since 1963. Severe contractures with scarring and adhesions began to incapacitate him in 1968. The patient denied dysphagia or problems with bowel movements. He has a strong family history for this disorder (Fig. I). I n 1971, an ulcer developed on his right lower leg, which was biopsied for possible squamous cell carcinoma. At that time. he had an excision and split-thickness skin graft. The biopsy showed pseudoepitheliomatous hyperplasia. Repeat biopsy at the time of referral showed squamous cell carcinoma. Several treatment modalities were employed, including Tubercidin, 5-fluorouracil ointment 20%, and purified protein derivative injection followed by debridement and skin grafting , with only temporary improvement. In 1978, a below-the-knee amputation was performed. Right inguinal nodes had been enlarged for years, but needle aspiration revealed only lymph node hyperplasia. Physical examination showed no abnormality except for the skin. The patient had widespread areas of blistering on his extremities and lower back, often in oval or nunimular pat-FIG. 1. Genealogy chart. Genealogy of family and appearance of e...

We report a 59-year-old woman with disease manifestations diagnosed as mycosis fungoides (MF), as well as findings of sarcoidosis. The concurrence of these two disorders, both of which may be associated with basic immunopathogenic factors, has been reported. Histologically, the cutaneous findings of both non-caseating granulomas and an infiltrate compatible with MF in the same site leads to a discussion of granulomatous MF. Whether granulomatous MF simply represents sarcoidosis developing concomitantly with MF lymphoma or is, in fact, a distinct clinical and histologic type of MF is discussed. Reasons why granulomatous MF, like granulomators Hodgkin disease, might have a more favorable prognosis than the usual type of MF are considered.

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