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.

Differentiation between merkel cell carcinoma and malignant melanoma: An immunohistochemical study.

BACKGROUND: Although Merkel cell carcinoma (MCC) exhibits specific clinical and histologic features, differentiation from other cutaneous neoplasms, such as lymphoma, metastatic oat cell carcinoma and malignant melanoma (MM), may sometimes be difficult. OBJECTIVE: The aim of our study was to immunohistochemically differentiate MCC from MM. METHODS: Paraffin sections from 6 cases of primary MCC and 6 cases of primary MM were investigated. For immunostaining, the APAAP method was used. RESULTS: Neuron-specific enolase was positive in all cases of MCC, as well as in 2 cases of MM. Marked positivity for cytokeratins 18, 20 and chromogranin A was observed in the MCC group, whereas a complete absence of expression of these three markers was noted in the MM group. Immunostaining with HMB45 and NKI/C3 was positive in all cases of MM and negative in all cases of MCC. S-100 protein was positive in all but 1 case of MM. In contrast, only 1 case of MCC reacted with S-100 protein. CONCLUSION: Our results underline the role of immunohistochemistry in the diagnosis and differential diagnosis of MCC. In particular, the combination of neuron-specific enolase, cytokeratins 18, 20 and chromogranin A positivity for MCC and HMB45, NKI/C3 and S-100 protein positivity for MM is of great value in the distinction between these two cutaneous neoplasms.

Experience with low-dose thalidomide therapy in chronic discoid lupus erythematosus.

BACKGROUND: Low-dose thalidomide therapy (median dose 100 mg/day, 50-200 mg/day) in chronic discoid lupus erythematosus was studied with regard to efficacy, tolerance, and toxicity in 22 patients. Intense contraceptive precautions were taken in women patients of childbearing age. METHODS: An open uncontrolled trial was conducted. Age, the total drug intake, disease duration, extent/severity, and adverse reactions were studied with regard to the final clinical outcome. The follow-up duration was 1.8 years (range 1 month to 3 years). RESULTS: With the exception of age (inverse correlation, P < 0.01), the parameters studied did not influence the final clinical amelioration: complete responders numbered 54.5%, partial responders 22.7%, and 13.6% were withdrawn from the trial with complaints of intolerance. The initial (first month) clinical response correlated significantly with the final one (P < 0.01). Drowsiness (40.9%) and somnolence (18.2%) were the most common side-effects, without affecting seriously the daily life of the participants. No case of real neurotoxicity was confirmed. Relapses occurred within 39.4 +/- 21.4 days after drug withdrawal, presenting a milder clinical picture. CONCLUSIONS: In the context of a predictable final outcome, low-dose thalidomide therapy is effective as an alternative choice in cases resistant to the usual treatment.

Immunohistochemistry in histoid leprosy.

BACKGROUND: Histoid leprosy is a rare form of multibacillary leprosy as the result of secondary or even primary resistance to dapsone. The etiopathogenesis has not been clarified up to now. METHODS: An immunohistochemical study was carried out for the expression of various markers on epidermal and dermal cell populations using sections of frozen skin specimens from 5 patients with histoid leprosy as compared to specimens from 7 tuberculoid and 7 lepromatous patients. RESULTS: Dendritic epidermal cells, identified by monoclonal antibodies against CD1, HLA-DR, CD45, and CD36, were found reduced in histoid leprosy as compared to both tuberculoid and lepromatous groups. A gradual reduction of keratinocytic HLA-DR expression from tuberculoid to lepromatous to histoid leprosy was observed. The pattern of CD36, CD4, and CD8 expression of lymphomonocytic cells in the dermis of histoid lesions was similar to that of tuberculoid leprosy, but without the formation of an organized granuloma. CD45+ cells as well as activated lymphocytic cells, expressed by the activation immunophenotype (CD1, HLA-DR, CD25, CD71, EGF-R) were found frequently in all groups. CONCLUSIONS: The in situ immunohistochemical findings support a modified hypersensitivity reaction of the cellular type that results in an inhibition of the lesional expansion, but not in the destruction of the bacilli within the histoid lesion.

Current profile of active leprosy in Greece; a five-year retrospective study (1988-1992).

The epidemiological characteristics of newly diagnosed, active leprosy cases (incidence, N = 16 Greeks and 4 expatriates) and relapsed cases (recurrences, N = 25, all Greeks) were studied. Most of the cases were multibacillary, over 50% being lepromatous. The relapses were analyzed by sex, disease duration and residence (rural or urban). Most of the newly diagnosed cases presented with nonreactional skin lesions (70%). The relapses were self-reported and detected mainly because of type 2 leprosy reactions (56%). The main source of the infection for new cases was members of their former extended family. The statistical trend of leprosy in Greece is a continuing decline in a country which already has a very low endemicity.