Mal de Meleda in Indonesia: Mutations in the SLURP1 gene appear to be ubiquitous.

Mal de Meleda is a rare autosomal recessive genodermatosis caused by mutations in the ARS B (SLURP1) gene, with possible founder effects in the Mediterranean and Adriatic regions. We report an affected individual from Indonesia without known consanguinity in the family, suggesting that SLURP1 gene mutations are ubiquitous. Recognition of the phenotype can be confirmed by genetic testing, thus facilitating genetic counselling.

Successful treatment of lymphomatoid papulosis with photodynamic therapy.

A 40-year-old woman presented with a prolonged history of recurrent crops of erythematous papules and nodules on her abdomen, arms and legs. Histological examination of a cutaneous biopsy revealed Type A lymphomatoid papulosis. Over a 3-year period, some of the patient's lesions had proven to be resistant to treatment with topical and intralesional corticosteroids and systemic agents including methotrexate, tetracycline and nicotinamide. These resistant lesions were treated with two sessions of methyl aminolevulinate photodynamic therapy given 1 week apart. Review 11 months post-photodynamic therapy demonstrated complete clinical clearance at the treatment site. While photodynamic therapy is considered a standard non-surgical treatment option for non-melanoma skin cancers and has been described in a number of non-oncological indications, this is the first report of its use in lymphomatoid papulosis.

Refractory subacute cutaneous lupus erythematosus successfully treated with rituximab.

A 48-year-old woman presented with pruritic, scaly, annular plaques over her upper back and chest that were clinically, serologically and histologically characteristic of subacute cutaneous lupus erythematosus (SCLE). She failed to respond to conventional treatment, which included high-dose hydroxychloroquine, methotrexate, prednisolone, chloroquine, acitretin, thalidomide, dapsone and azathioprine. Subsequently treated with intravenous rituximab 375 mg/m(2) weekly for 4 weeks, she remained on adjuvant oral hydrochloroquine 600 mg daily and topical clobetasol propionate 0.05% ointment as required. Clearing of annular plaques was noted 8 weeks after the initial course of rituximab. By 12 weeks there were no new lesions and only post-inflammatory hyperpigmentation remained. Both hyper- and hypopigmentation, which is more common, are consistent with SCLE lesion regression. Skin lesions recurred 11 months later; however, no further lesions occurred after re-introduction of rituximab therapy. The treatment was well tolerated. A maintenance regimen of rituximab, 375 mg/m(2) every 8 weeks for 2 years, was commenced 3 months after completing the second course of treatment, with ongoing disease remission. Rituximab appears to have activity in refractory SCLE and clinical trials are required to further assess this potential therapy.

Short-course therapy with imiquimod 5% cream for solar keratoses: a randomized controlled trial.

A dual-centre, randomized, double-blind, vehicle-controlled study was conducted to evaluate the safety and efficacy of short courses of therapy with imiquimod 5% cream in clearing >/=75% of baseline solar keratoses (SK) within a field of treatment. Subjects with 5-15 baseline SK within one treatment area (scalp, forehead and temples, or both cheeks) were randomized to apply imiquimod or vehicle cream to the entire treatment area three times a week for 3 weeks. Subjects were assessed 4 weeks after completing the first course for clearance of lesions. Subjects with <75% clearance were commenced on a second 3-week course of study cream. Subjects with >/=75% clearance were followed up until study completion without further therapy. All subjects were evaluated at the study endpoint of 14 weeks after initiating therapy for assessment of the primary outcome (>/=75% clearance of baseline solar keratoses). Twenty-one out of 29 (72%) imiquimod-treated subjects cleared >/=75% of baseline lesions compared with 3/10 (30%) subjects using the vehicle cream (Fisher's exact test, P = 0.027). Imiquimod was well tolerated. The present study has a short follow-up endpoint, but suggests that imiquimod is a potential therapeutic alternative in patients with SK.

Chronic actinic dermatitis: a retrospective analysis of 44 cases referred to an Australian photobiology clinic.

A retrospective study was performed to analyse the clinical and photobiological features and therapeutic outcomes of 44 patients with chronic actinic dermatitis who were evaluated over an 8.3-year period. The study population comprised 37 men and seven women with a mean age of 62.7 years (range 26-85 years). The most common abnormal phototest results were decreased minimal erythema doses to both UVA and -B (73.8%), and to UVA alone (14.3%). Twenty-six patients (78.8%) had at least one allergic, photoallergic or combined allergic/photoallergic reaction. A total of 139 positive contact or photocontact reactions were recorded (mean 4.2 per patient). Most commonly, treatment consisted of photoprotection, topical corticosteroids and episodic use of systemic agents, in particular azathioprine.

Clinical study of primary cutaneous B-cell lymphoma using both the European Organization for Research and Treatment of Cancer and World Health Organization classifications.

Primary cutaneous B-cell lymphoma (PCBCL) is rare, with few series reported in the literature. Its classification and treatment remain controversial. Biopsy specimens of 13 patients with PCBCL were classified according to both the European Organization for Research and Treatment of Cancer (EORTC) and the new World Health Organization (WHO) classifications. Treatment and clinical outcomes were documented. Using the EORTC classification there were seven men and six women aged 32-85 years (mean = 51 years) with follicle centre cell (FCC) lymphoma (nine), immunocytoma (two) and primary cutaneous large B-cell lymphoma of the leg (PCLBCL-leg) (two). When the WHO classification was used, the nine patients with FCC were reclassified as follicle centre (five) and diffuse large B-cell lymphoma (four). Most patients had localized disease (12). Initial treatment consisted of radiotherapy alone (seven), combination chemotherapy alone (one), combined chemoradiotherapy (three) and surgery (two). Twelve patients achieved complete remission (median follow up 28 months, range 10-167 months). One patient with PCLBCL-leg died from progressive cutaneous disease. Most localized PCBCL lesions (except PCLBCL-leg) have a favourable prognosis. We recommend that clinicians be familiar with the important differences in the EORTC and WHO classifications. Further large prospective studies comparing the WHO and EORTC classifications are required to more clearly delineate the outcomes of the increasing number of patients who are classified as DLBCL by the WHO system.