Narrow-band UVB phototherapy and psoralen-ultraviolet A photochemotherapy in the treatment of cutaneous mastocytosis: a study in 20 patients.

BACKGROUND: In mastocytosis, the skin is almost invariably involved, and cutaneous symptoms deeply affect patients' quality of life. METHODS: A retrospective observational analysis of patients affected by cutaneous mastocytosis (CM) and indolent systemic mastocytosis (ISM) treated with phototherapy/photochemotherapy (PUVA or NB-UVB) has been conducted. For each patient, total numbers of PUVA or NB-UVB exposures, the cumulative UV dose (J/cm2 ), serum tryptase profile, and pruritus, before and after treatment, according to the visual analogue scale (VAS) were considered. Skin lesions of each patient were assessed, before and after treatment, according to a cutaneous scale score. RESULTS: Twenty patients affected by CM and ISM were studied; in particular, 10 patients received NB-UVB therapy, and other 10 patients received PUVA. A statistically significant mean reduction of pruritus in both groups (P < 0.01) was observed. The number of treatments necessary to obtain symptom relief was significantly lower in the PUVA group, but the mean exposure dose was significantly higher, if compared to the NB-UVB group. Serum tryptase levels showed a downward trend. The cutaneous score improved in both groups. LIMITATIONS: This study was a retrospective study with a small sample size and without a control group. CONCLUSION: This work provides evidence that both NB-UVB and PUVA represent a safe and useful second-line therapy of the cutaneous symptoms in mastocytosis.

Telangiectasia macularis eruptive perstans successfully treated with PUVA therapy.

We present the case of a 58-year-old woman who was diagnosed as having telangiectasia macularis eruptiva perstans (TMEP) and was successfully treated with PUVA photochemotherapy. During the 6-month follow-up, no recurrence of pruritus or skin lesions was observed. TMEP represents a rare form of cutaneous mastocytosis, which is clinically characterized by reddish-brown telangiectatic macules symmetrically distributed over the trunk and extremities. Although in the majority of cases the disease is limited to the skin, systemic involvement may occur. The treatment of TMEP is challenging and several therapeutic modalities have been proposed in the past.

Anetodermic mastocytosis: response to PUVA therapy.

Mastocytosis is a group of disorders characterized by the accumulation of mast cells in different tissues and organs. The skin is the most frequently involved organ (90% of cases) where mastocytosis may show a heterogenic clinical expression. Anetodermic lesions are an unusual clinical presentation of mastocytosis. We report a case of anetodermic mastocytosis in a 26-year-old man, with sparse lesions and a benign course. PUVA therapy obtained excellent results in this case. Darier's sign should be investigated in patients with anetodermic lesions of unclear origin in order to exclude cutaneous mastocytosis.

Telangiectasia macularis eruptiva perstans with an associated myeloproliferative disorder.

Telangiectasia macularis eruptiva perstans (TMEP) is a cutaneous form of mastocytosis. It has been rarely associated with an underlying myeloproliferative disorder. We report the case of a patient, while receiving treatment for thrombocytosis, with both platelet production and function inhibitors presented with TMEP. TMEP is often refractory to therapy; however, our patient responded to treatment with PUVA.