Narrowband (TL-01) ultraviolet B phototherapy for pruritus in polycythaemia vera.

BACKGROUND: There are several reports of the efficacy of broadband ultraviolet (UV) phototherapy in the treatment of pruritus associated with polycythaemia vera. OBJECTIVES: To evaluate whether narrowband (TL-01) UVB phototherapy is also effective in treating this condition. METHODS: Ten patients with pruritus associated with polycythaemia vera were treated with narrowband (TL-01) UVB phototherapy. The first irradiation dose was 2/3 of the minimal erythema dose; the treatment schedule consisted of three irradiation sessions per week, with dose increments of 10% each session for skin types I and II, and 15% for skin types III and IV. RESULTS: Patients reported a marked relief of symptoms after an average of six treatments (median cumulative dose 1851.52 mJ cm-2, range 1180.4-2468.4). A complete remission of the pruritus occurred within 2-10 weeks of treatment (median cumulative dose 5371.46 mJ cm-2, range 3271.2-7336.3) in eight of 10 patients. Two patients had only a partial and temporary relief of pruritus after two cycles of treatment and a cumulative dose of 3271.2 mJ cm-2. CONCLUSIONS: Narrowband UVB phototherapy is effective for treatment of pruritus associated with polycythaemia vera, and has the advantage of being less erythemogenic than broadband UVB.

Marie Unna hereditary hypotrichosis.

Hereditary congenital hypotrichosis is an autosomal dominant pilar dysplasia first described by Marie Unna in an extended German family. The diffuse hair defect typically occurs as an isolated phenomenon and the ultrastructural hair findings consist of both torsion and longitudinal grooving of the hair shaft. A large pedigree comprising 6 generations with 20 members affected by Marie Unna hypotrichosis from Italy is reported.

Hereditary multiple fibrofolliculomas, trichodiscomas and acrochordons: syndrome of Birt-Hogg-Dubè.

The syndrome of Birt-Hogg-Dubè (BHD) is an autosomal dominant syndrome, characterized by a triad of cutaneous lesions including multiple fibrofolliculomas, trichodiscomas, and acrochordons. There are many clinical expressions, solitary and multiple forms, with or without other skin tumors. In the literature BHD syndrome has been associated with internal malignancy. We describe a patient with multiple firm, skin-colored papules in which the three types of lesion are documented. No signs of systemic disease were present.

Findings in vivo of Sarcoptes scabiei with incident light microscopy.

Parasites, especially Sarcoptes scabiei, may represent a difficult diagnostic problem. In many cases, the results of the usual methods for the diagnosis of scabies, such as microscopic identification, can be negative owing to the small number of parasites present in the cornified layer. Moreover, tape stripping or removal of mites with a needle is difficult and sometimes painful, and therefore, not well accepted. We have evaluated the applicability and usefulness of epiluminescence microscopy, a new technique frequently used for the study of pigmented lesions of the skin. Thirty seven patients with the presumptive clinical and/or anamnestic diagnosis of scabies were enrolled in our study. With this method many suspicious sites may be examined within a few minutes without causing any harm or discomfort to the patient.

Epiluminescence microscopy for the diagnosis of doubtful melanocytic skin lesions. Comparison of the ABCD rule of dermatoscopy and a new 7-point checklist based on pattern analysis.

OBJECTIVE: To compare the reliability of a new 7-point checklist based on simplified epiluminescence microscopy (ELM) pattern analysis with the ABCD rule of dermatoscopy and standard pattern analysis for the diagnosis of clinically doubtful melanocytic skin lesions. DESIGN: In a blind study, ELM images of 342 histologically proven melanocytic skin lesions were evaluated for the presence of 7 standard criteria that we called the "ELM 7-point checklist." For each lesion, "overall" and "ABCD scored" diagnoses were recorded. From a training set of 57 melanomas and 139 atypical nonmelanomas, odds ratios were calculated to create a simple diagnostic model based on identification of major and minor criteria for the "7-point scored" diagnosis. A test set of 60 melanomas and 86 atypical nonmelanomas was used for model validation and was then presented to 2 less experienced ELM observers, who recorded the ABCD and 7-point scored diagnoses. SETTINGS: University medical centers. PATIENTS: A sample of patients with excised melanocytic lesions. MAIN OUTCOME MEASURES: Sensitivity, specificity, and accuracy of the models for diagnosing melanoma. RESULTS: From the total combined sets, the 7-point checklist gave a sensitivity of 95% and a sepcificity of 75% compared with 85% sensitivity and 66% specificity using the ABCD rule and 91% sensitivity and 90% specificity using standard pattern analysis (overall ELM diagnosis). Compared with the ABCD rule, the 7-point method allowed less experienced observers to obtain higher diagnostic accuracy values. CONCLUSIONS: The ELM 7-point checklist provides a simplification of standard pattern analysis because of the low number of features to identify and the scoring diagnostic system. As with the ABCD rule, it can be easily learned and easily applied and has proven to be reliable in diagnosing melanoma.