UVA1 phototherapy for cutaneous diseases: an experience of 92 cases in the United States.

BACKGROUND: The efficacy and safety of UVA1 (340-400 nm) phototherapy were established by studies from European countries. PURPOSE: Evaluate experience with UVA1 phototherapy for patients with cutaneous diseases in the United States. METHODS: A retrospective analysis of 92 cases of UVA1-treated cutaneous conditions from four medical centers in the United States was performed. RESULTS: Two-third of the patients showed a fair to good response (26-100% improvement) and one-third of the patients showed a poor response (0-25% improvement). Diseases with a moderate to good response (51-100% improvement) included scleredema adultorum, hand or foot dermatitis, atopic dermatitis, morphea (medium or medium- to high-dose UVA1), systemic sclerosis, and urticaria pigmentosa. Besides tanning, other adverse effects were found in 15% of patients, which include pruritus, erythema, tenderness, and burning sensation. Patients with skin types I-III responded better that those with a darker skin type. CONCLUSION: UVA1 phototherapy is a useful and well-tolerated treatment option for a variety of skin conditions.

[Multiple fibromas in systemic mastocytosis]. / Multiple Fibrome bei systemischer Mastozytose.

An adult man with systemic mastocytosis developed multiple fibromas within his involved skin, predominantly in the intertriginous areas. Friction in the intertriginous areas and scratching due to severe itch may have induced the release of mast cell factors which subsequently resulted in fibroma formation.

[Coexistence of pigmented urticaria and mastocytomas: a case report]. / Wspólistnienie pokrzywki barwnikowej ze zmianami typu mastocytoma--opis przypadku.

Mastocytoses are a heterogeneous group of disorders of unknown etiology characterised by the accumulation of mast cells, particularly in the skin. Mastocytosis may be limited to the skin or involve internal organs, especially those playing the lymphoreticular function. We present a case of 9-year-old girl, with a history of maculopapules at the age of two, followed by yellow-brownish, nodular skin lesions. The skin lesions, located on the trunk, neck and face were accompanied by pruritus. The histopathological examination of the node confirmed the diagnosis of mastocytoma. A bone scintigram showed an increased uptake of radiotechnetium around the right coxal region. Application of antihistaminic drugs and phototherapy (UVA) has led to partial remission of skin manifestations of the disease. A long-term follow-up of the child is necessary.

Short- and long-term effectiveness of oral and bath PUVA therapy in urticaria pigmentosa and systemic mastocytosis.

BACKGROUND: Previous studies have shown that oral PUVA is effective in urticaria pigmentosa. Long-term results, however, are unknown. OBJECTIVE: We studied the long-term effectiveness of oral PUVA treatment in urticaria pigmentosa as well as in systemic mastocytosis. In addition, the success of bath PUVA was examined in these diseases. METHODS: Twenty patients with urticaria pigmentosa and systemic mastocytosis treated by oral PUVA were examined retrospectively for a time period of up to 18 years. We studied the duration of improvement and correlated these results with the total PUVA dose, the skin type and the age of onset. Four patients were treated by bath PUVA therapy. RESULTS: In oral PUVA therapy an improvement was seen in 14 out of 20 patients (70%). There was no difference in the response rate between urticaria pigmentosa and systemic mastocytosis and there was no correlation with the total PUVA dosage. The duration of the treatment's success ranged from a few weeks to more than 10 years. 25% of the patients showed an improvement for more than 5 years. Patients with onset during childhood and early adolescence and patients with skin types I and II responded favourably to the treatment. Bath PUVA therapy was without effect in our 4 patients. CONCLUSION: Oral PUVA is very effective for the long-term treatment of urticaria pigmentosa as well as systemic mastocytosis.

Photochemotherapy of dominant, diffuse, cutaneous mastocytosis.

Diffuse, cutaneous mastocytosis is a rare variant of cutaneous mast cell infiltration that can arise in neonates or infants as a generalized bullous eruption. The mode of transmission is suggested as autosomal dominant. We report four infants from two unrelated families with diffuse cutaneous mastocytosis whose cutaneous disease was not controlled by initial therapies. Treatment of the four infants with photochemotherapy dramatically reduced or eliminated symptoms. One course of therapy resulted in improvement, and retreatment has not been required two to six years later.

[Urticaria pigmentosa adultorum and systemic mastocytosis--diagnostic principles and therapeutic possibilities]. / Urticaria pigmentosa adultorum und systemische Mastozytose--Diagnostische Grundzüge und therapeutische Möglichkeiten.

We report on the clinical picture of urticaria pigmentosa adultorum and systemic mastocytosis. In four out of five patients, we proved systemic mastocytosis by means of a biopsy from the iliac crest, although these patients did not complain of systemic signs and symptoms. In all cases, the cutaneous changes and clinical symptoms could be improved by a four-week-heliotherapy in the North Sea summer climate; itching did not reappear but five months later. Heliotherapy is regarded as an useful alternative concerning the treatment of urticaria pigmentosa and cutaneous manifestation of systemic mastocytosis.

The spectrum of mastocytosis.

Mastocytosis represents a spectrum of clinical disorders that results from an aberrant proliferation of tissue mast cells. This disease process may be confined to the skin (cutaneous mastocytosis) or may involve multiple organs (systemic mastocytosis). Parameters that are useful in differentiating cutaneous from systemic disorders include patient age, symptom complex, and clinical signs. A wide range of clinical symptoms may be encountered in patients with mastocytosis which result from the release of pharmacologically potent mast cell mediators. Distinct cutaneous patterns resulting from skin mast cell infiltrates can be helpful in identifying patients with systemic involvement. The diagnosis of mastocytosis is confirmed by demonstrating increased tissue mast cells in involved organs. The overall prognosis for patients with proliferative mast cell disease is relatively good, although a small percentage are at risk for developing a fatal neoplastic disorder (malignant mastocytosis). Treatment of mastocytosis is directed at both inhibiting mast cell degranulation and blocking the potential systemic effects of released secretory products. Future therapeutic advances depend upon an improved understanding of the basic mechanisms involved in mast cell mediator release and the forces that govern mast cell growth and development.

Urticaria pigmentosa responsive to nifedipine.

A patient with symptomatic urticaria pigmentosa who responded to nifedipine therapy is reported. Relief from cold-induced urtication and flushing was obtained with 10 mg taken orally three times daily. Calcium influx is an early step in the degranulation of mast cells. We hypothesize that the beneficial effect of nifedipine was due to calcium-channel blockade causing elevation of the mast cell threshold for degranulation.

Response of cutaneous mast cells to PUVA in patients with urticaria pigmentosa: histomorphometric, ultrastructural, and biochemical investigations.

In six patients with urticaria pigmentosa, population density and ultrastructure of the cutaneous mast cells and histamine levels of the lesional skin were studied before, immediately after, and again 5-8 months after photochemotherapy (PUVA). Immediately after PUVA, the total mast cell number was not reduced, but on separate analysis of the intradermal distribution, significantly fewer mast cells were found in the papillary dermis and correspondingly more mast cells in the adjacent upper dermis. On electron microscopic examination, 4% of the mast cell granules were immature before and 27% after PUVA therapy, based on the lower electron density of the granular matrix. This was associated with a markedly lower histamine content of the lesional skin. Five to eight months after recovery from PUVA, the morphologic changes and the histamine levels had all returned to the pre-PUVA status. These findings were paralleled by a reversal of all clinical beneficial effects that had been observed with PUVA.

Photochemotherapy (PUVA) in the treatment of urticaria pigmentosa.

Eight patients received PUVA for mastocytosis. Five women had typical adult-onset urticaria pigmentosa, without evidence of systemic disease. Another woman had suspected hepatic involvement while the remaining female had early-onset familial urticaria pigmentosa with morphologically atypical mast cells. The only male patient had cirrhosis with hepatic deposits of mast cells in addition to polycythaemia rubra vera. In all patients, except the man with systemic disease, there was reduced pruritus and wealing and partial to almost complete fading of the macules. The manifestations of urticaria pigmentosa recurred after treatment was discontinued. In both lesional and uninvolved skin there was no significant change in either the mean mast cell counts or mast cell ultrastructure after an average of twenty-seven PUVA exposures. In addition, PUVA did not cause a significant alteration in the histamine content of the skin. The beneficial effect of PUVA in urticaria pigmentosa therefore does not appear to be directly related to a change in mast cell numbers or morphology, or to the histamine concentration in the skin.

Mastocytosis and the mast cell.

Mastocytosis is a disease in which the mast cell population is increased in various tissues. Although the mechanism for the increased density of mast cells is not understood, recent research into mast cell structure and function has improved our understanding of the symptomatology and prompted newer and better approaches to treatment. To be discussed, and of particular interest to the dermatologist, is the management of, and evaluation for, systemic disease in a patient presenting with cutaneous findings.

[Dermal mastocytes, histaminaemia, and oral photochemotherapy (author's transl)]. / Mastocytes cutanés, histaminémie et photochimiothérapie orale.

One patient with urticaria pigmentosa was treated with PUVA. This treatment resulted in generalized tanning, decrease of Darier's sign and improvement of pruritus. However, the hyperpigmented macules overlying the mast cell infiltrates showed some persistence. This patient was subjected to biopsy of lesional skin before, during and at the end of oral photochemotherapy. During and after PUVA-therapy, histological examination revealed an increased number of free granules and a progressive decrease in the number of dermal mastocytes. Ultrastructurally necrotic cellular changes in the dermal mastocytes were observed. This suggests that PUVA-therapy may exercise its beneficial effect in urticaria pigmentosa by direct destruction of these cells. In addition, PUVA stimulates mast cell degranulation. Histaminaemia was estimated in this patient, before and after several irradiations. No significant changes were observed. Furthermore, histaminaemia was assayed in 15 patients with various dermatoses undergoing PUVA-therapy. No significant changes were observed. It is concluded that histaminaemia assayed using the fluorometric method of Shore et al. remains unchanged during oral photochemotherapy.