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...

Fourteen patients with Kaposi's sarcoma (KSY were treated systemically with vinblastine sulfate in a low-dose regimen and compared with 23 patients reported in the medical literature. The therapeutic results in our series were excellent in terms of regression of cutaneous lesions. Vinblastine appears to be a drug that is well suited for the management of KS in an outpatient setting. Intravenous therapy may be supplemented with intralesional or intraarterial vinblastine.

Two cases of squamous cell carcinoma developing in lesions of chronic cutaneous discoid lupus erythematosus are presented. One of the patients was white and the other was black. The squamous cell carcinoma in the black patient proved to be rapidly metastatic and eventually fatal. The white patient developed seven squamous cell carcinomas over 8 years with no evidence of metastatic spread. The role of ultraviolet light in inducing skin cancer in these patients is discussed.

Our studies have been carried out in over 500 patients with an aggregate of more than 15,000 lesions for observation periods in excess of 5 years. Topical chemotherapy with 5-FU was shown to result in selective eradication of premalignant and superficial malignant lesions of the skin. Since the action of 5-FU is selective, it produces minimal or no effects on normal tissues adjacent to tumors. Healing therefore occurs with slight or no apparent scarring. Topical chemotherapy is indicated for solar keratoses and other precancerous lesions as well as for multiple superficial primary skin cancers when standard therapeutic measures are not suitable because large areas of the skin are involved by persistently developing tumors. Topical cancer chemotherapy induces the resolutionof premalignant skinlesions and prevents their further development to the malignant stage. Early primary skin cancers and premalignant lesions react to topical antitumor agents before they are otherwise clinically detectable. These tumors can therefore be eradicated before they present significant clinical problems. Early detection and eradication of skin cancers and premalignant skin lesions furthermore result in the reduction of the subsequent incidence of clinically significant tumors. Local chemotherapy therefore appears to have prophylactic value. Local administration of 5-FU results in high concentrations of the antitumor agent at the tumor site, although the total amount administered and the rate of absorption are relatively low. General toxicity such as bone marrow depression and other systemic side-effects are therefore not encountered with local cancer chemotherapy. Superficially located neoplasms are suitable model systems for the study of tumor biology. Local chemotherapy indicates that differential and selective chemical effects can be produced in neoplastic cells and their normal counterparts. Studies on local chemotherapy demonstrate that the selective eradication of malignant disease in many by chemical means is feasible.

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