Epidermolysis bullosa acquisita (EBA) is a rare autoimmune bullous disorder that is often difficult to treat. Few cases have been reported and therapy consists mainly of combinations of systemic steroids, immunosuppressants and, recently, administration of intravenous human immunoglobulin (IVIg). We describe a case of EBA in which our therapeutic choices were limited due to the patient's poor general condition, including extensive infection of the lesions and a history of pulmonary tuberculosis. The patient was treated with IVIg at a dose of 400 mg/kg per day for 5 consecutive days every 4 weeks. The treatment was well tolerated and the results were satisfactory. It seems that IVIg, due to its possible immunomodulatory mode of action, can be an efficacious therapeutic agent in this rare autoimmune disease.
During the topical treatment of 45 patients, who had extensive forms of alopecia areata, with the allergen diphencyprone, 3 of them (6.7%) developed vitiligo. Two were females and 1 male aged 53, 19 and 28 years respectively. None of these patients had a personal or family history of vitiligo. Vitiligo appeared 3–5 months after the onset of treatment and was localized only to the areas of topical application in the younger woman and the man. In the older woman, vitiligo extended to several areas apart from those where the medicament was applied. After the end of diphencyprone treatment, vitiligo had a spontaneous significant improvement only in the man. Mitochondrial autoantibodies were found in the older woman only. To our knowledge, vitiligo due to diphencyprone has not been previously reported.
Hereditary sensory neuropathies comprise a group of rare childhood diseases which are classified into four types. We present a Greek boy 11 years old with hereditary sensory neuropathy type IV (congenital sensory neuropathy with anhidrosis) whom we have followed up and studied during the last seven years. Our patient presented for the first time with recurrent hyperthermic episodes without sweating, and lack of pain sensation from the first months of life. Insensitivity to pain and thermal stimuli had resulted in burns on the extremities and self-mutilation of the tongue, lips and fingertips. When he was five and seven years old respectively he had two painless fractures of the ankles which led to insoluble orthopedic problems. He also suffered from mental retardation, which was obvious from his first years of life. Sweat gland investigations showed significant hypohidrosis or anhidrosis although the sweat glands were normal microscopically. Hereditary sensory neuropathy type IV, although rare, is important for dermatologists because it must be differentiated from other anhidrotic syndromes, and in view of the poor prognosis of the condition.
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