Authors report herein a case of a 27-year-old male patient who had been suffering from chronic ulcerative dermatitis with scar-like changes and successive involvement of the ankles, limb folds, nape and abdomen, and no tendency to heal from age 4. At the age 14, an immunologic study showed a selective IgA deficiency with partial T-lymphocyte inactivation. Ten years later, at age 24, he showed a severe form of morphea over a generalized area and disabling joint contractures, and was diagnosed as disabling pansclerotic morphea with an IgA value that returned to a near normal level. At the age 27, an adult-fist, 7×6×4cm sized, squamous cell carcinoma with an easy bleeding tendency like an overgrowing granulation tissue vascular tumor had developed on the chronic ulcerative lesion on the posterior aspect of the right ankle for 2 months. Aggressive metastatic lesion occurred on the right popliteal area 3 months later. He died 1 month thereafter.