Cutaneous mastocytosis: demographic aspects and clinical features of 55 patients.

BACKGROUND: Mastocytosis is a rare, heterogeneous group of disorder with abnormal increase of mast cells in one or more organ systems. OBJECTIVE: To evaluate the demographic and clinical features of cutaneous mastocytosis (CM). METHODS: Records of 55 patients with cutaneous mastocytosis were retrospectively analysed. RESULTS: Of the 22 females and 33 males, 80% had urticaria pigmentosa/maculopapular CM and 20% had mastocytoma. Of all cases, 81.8% had first lesions in childhood. The most common presentation was involvement of trunk together with extremities. Thirteen (23.6%) patients had history of bulla; Darier's sign was positive in 34 of 38 patients. Itching was the most common complaint, provocated by hot weather/bath. CONCLUSION: Clinical presentations of urticaria pigmentosa/maculopapular CM and mastocytoma are similar regarding gender, age of onset, age of diagnosis, and presence of Darier's sign and history of bulla. In contrast to mastocytoma, urticaria pigmentosa/maculopapular CM lesions were frequently located on trunk together with extremities.

Experience with calcipotriol as adjunctive treatment for vitiligo in patients who do not respond to PUVA alone: a preliminary study.

BACKGROUND: PUVA therapy remains a primary treatment for vitiligo, despite unsatisfactory results. Because of calcipotriol's reported effects on melanocytes and on immunomodulatory and inflammatory mediators we wondered whether adding calcipotriol to PUVA would be more effective than PUVA alone in treating vitiligo. OBJECTIVE: We sought to determine whether the combination of topical calcipotriol and PUVA therapy increases the responsiveness of patients with vitiligo refractory to PUVA alone. METHODS: Twenty-one patients with vitiligo refractory to previous PUVA therapy were studied. Patients received 60 sessions of PUVA 3 times a week and 0.005% topical calcipotriol twice daily. Patients were monitored for repigmentation overall and on the trunk, extremities, and acral regions. RESULTS: Starting at the median of the 17th treatment session, some degree of repigmentation was observed in 71.5% of the patients. After treatment, cosmetically acceptable overall repigmentation was observed in 29% of patients; repigmentation of lesions on the trunk, extremities, and acral region was noted in 36%, 58%, and 0% of patients, respectively. Adverse reactions were mild and tolerable. CONCLUSION: The combination of PUVA and calcipotriol may be effective therapy and should be further investigated for the treatment of vitiligo.

Serum adenosine deaminase levels in patients with psoriasis: a prospective case-control study.

Adenosine deaminase (ADA) activity is a nonspecific marker of T cell activation. T cell activation is thought to play an important role in the pathogenesis of psoriasis. Our purpose was to assess the significance of serum ADA activity in psoriasis and its relevance to disease activity. ADA activity was determined with an enzymatic method in 25 patients with psoriasis and in 15 healthy subjects. These measurements were repeated for 10 patients after either PUVA or cyclosporin A treatments. Disease activity was estimated by the PASI scoring system. Serum ADA level was significantly elevated in patients with psoriasis compared to healthy subjects (p<0.05). There was a significant decrease in the ADA levels after treatment compared to pretreatment values in the same patients (p<0.05). There was no correlation between ADA levels and PASI scores. These results support the evidence that T cell activation is involved in the pathogenesis of psoriasis and that ADA may be valuable in the assessment of disease activity in psoriasis.

Kindler syndrome: absence of definite ultrastructural feature.

Kindler syndrome is characterized by congenital blister formation, photosensitivity, poikiloderma, and cutaneous atrophy in later life. There are few reports about the ultrastructural features of this syndrome, but still there is no consensus about the basic disease. Here we report a case of Kindler syndrome with ultrastructural findings.