Follicular mucinosis associated with early stage cutaneous T-cell lymphoma: successful treatment with interferon alpha-2b and acitretin.

BACKGROUND: Follicular mucinosis (FM) is a rare dermatosis characterized by mucin deposits in the pilosebaceous units. It is divided into a primary-benign type and a secondary type associated mostly with lymphomas. No standard effective therapy is available for the primary FM while in the secondary form treatment is aimed against the underlying disease. METHODS: We report a case of secondary FM in which a cutaneous T-cell lymphoma was detected 6 years after the initial eruption. RESULTS: Complete remission was achieved with combination therapy of interferon alpha-2b at a dose of 6 million U subcutaneously three times a week, and acitretin 35 mg/day, for 6 months. CONCLUSION: Regular clinical and histopathological evaluation is suggested for all patients with FM. For cases associated with cutaneous T-cell lymphoma the combination of interferon alpha and acitretin seems to be a good therapeutical approach.

Treatment of Darier's disease with photodynamic therapy.

BACKGROUND: Photodynamic therapy (PDT) using topical 5-aminolaevulinic acid (5-ALA) as a photosensitizer has been reported in the treatment of both neoplastic and benign cutaneous disorders. OBJECTIVES: To evaluate the efficacy of photodynamic therapy in selected patients with Darier's disease (keratosis follicularis). METHODS: Six patients with Darier's disease were assessed before and after treatment with PDT using 5-ALA and mean fluence rates of 110-150 mW cm-2. RESULTS: Of the six patients, one was unable to tolerate the treatment. Of the remaining five, all experienced an initial inflammatory response that lasted two to three weeks. In four of the five patients, this was followed by sustained clearance or improvement over a followup period of six months to three years. Three of these four patients were on systemic retinoids and the fourth had discontinued acitretin prior to PDT. In the fifth patient partial improvement was followed by recurrence after etretinate therapy was discontinued. Biopsy specimens taken immediately after the procedure in two patients demonstrated a mild inflammatory cell infiltrate in the dermis. A biopsy obtained eighteen months after PDT from a successfully treated area showed no signs of Darier's disease and a subtle increase of collagen in the upper dermis. CONCLUSIONS: Photodynamic therapy can be viewed as a potential adjunctive modality for Darier's disease but should not be considered as a substitute for retinoids in patients who require systemic treatment.

Epidermolysis bullosa acquisita: treatment with intravenous immunoglobulins.

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.