[Scleredema adultorum Buschke and exulcerated necrobiosis lipoidica in diabetes mellitus type 1]. / Scleroedema adultorum Buschke und exulzerierte Necrobiosis lipoidica bei Diabetes mellitus Typ 1.

HISTORY AND ADMISSION FINDINGS: A 53-year-old woman suffering from brown plaques occasionally ulcerating at both lower legs and in the course of disease on the back of the foot presented at our department. Moreover she complained about an increasing sclerosis of her skin at the back over the last 15 years. She had suffered from diabetes mellitus type-1 for more than 40 years. INVESTIGATIONS: Skin biopsies form the ulcerated plaques showed changes typical for necrobiosis lipoidica. Biopsies of the back were diagnosed as scleredema adultorum Buschke. Laboratory tests displayed a normal antinuclear antibody titer. The differential diagnosis of systemic sclerosis could be ruled out. TREATMENT AND COURSE: We repeatedly performed debridement of ulcers, cream-PUVA therapy, applied a vacuum-sealing and topical corticosteroids at the lower legs. Meanwhile she received oral pentoxyfillin. The scleredema was treated with UVA-1 phototherapy but had to be stopped for high photosensitivity. Additionally physiotherapy was prescribed. Nonetheless the course of disease was chronic and therapy-resistant. CONCLUSION: Skin diseases are common in diabetes mellitus. Necrobiosis lipoidica and scleredema adultorum Buschke are rare complications but often refractory to treatment.

Dramatic response of chronic ulcerating necrobiosis lipoidica to ultraviolet A1 phototherapy.

We report on a 52-year-old female patient with chronic, ulcerating necrobiosis lipoidica (NL) who dramatically responded to ultraviolet A1 (UVA1) phototherapy. The patient had NL on her right shin for more than 30 years without evidence of diabetes mellitus. Treatment with a variety of local and systemic agents failed to prevent progression into ulceration, which necessitated repeated surgical interventions in the past. When the patient presented again with torpid ulcers at the periphery of previously grafted skin, high-dose UVA1 phototherapy was initiated. Improvement occurred rapidly and after 22 irradiations and a total exposure dose of 1480 J/cm², the ulcers had healed completely. During a follow-up period of 6 years,two minor recurrences were successfully retreated with UVA1 phototherapy.

Treatment of necrobiosis lipoidica with topical psoralen plus ultraviolet A.

BACKGROUND: Necrobiosis lipoidica (NL) is a rare skin disease, mostly seen on the legs and often occurring in patients with diabetes mellitus. The disease belongs to the idiopathic cutaneous palisading granulomatous dermatitides associated with a degeneration of collagen, thus leading to skin atrophy. Application of topical corticosteroids is the most widely used treatment but the results are not always satisfactory and may worsen skin atrophy. Preliminary studies in patients with NL have shown a clinical response with psoralen plus ultraviolet (UV) A (PUVA). Objectives To study the effect of topical PUVA on NL in a multicentre prospective study. METHODS: Thirty patients (27 women and three men) including 13 with insulin-dependent diabetes mellitus, with a diagnosis of NL proven by histopathology, were included. All patients had been unsuccessfully treated with topical and/or intralesional corticosteroids. Patients were treated twice weekly with an aqueous gel containing 0.005% psoralen followed by irradiation with UVA. Clinical photographs were taken for evaluation. In addition, 20-MHz high-frequency ultrasound analysis was performed in 10 patients to evaluate the thickness and density of the dermis during topical PUVA therapy. RESULTS: Five patients (17%) showed complete clearing (healing of ulceration and disappearance of erythema) after a mean of 22 exposures (range 15-30). Eleven patients (37%) showed improvement, defined as healing of ulceration and/or reduction of erythema, after a mean of 23 exposures (range 11-42). Ten patients (33%) showed no effect and four patients (13%) worsened during topical PUVA therapy. The treatment results of the patients who suffered from diabetes mellitus were not different from those who did not have diabetes mellitus. No difference was seen in mean dermal thickness (1666 vs. 1706 micro m) and density (17 vs. 16 units) before and after topical PUVA therapy. Side-effects were seen in 10 patients: hyperpigmentation (n = 4), blistering (n = 4) and bacterial infection (n = 2). CONCLUSIONS: Topical PUVA may be a useful treatment modality for NL in patients not responding to topical or intralesional corticosteroids.

High dose nicotinamide in the treatment of necrobiosis lipoidica.

An open study of high dose nicotinamide in the treatment of 15 patients with necrobiosis lipoidica is reported. Of 13 patients who remained on treatment for more than 1 month, eight improved. Improvement took the form of a decrease in pain and soreness, a decrease in erythema and the healing of ulcers if present, although the skin did not return completely to normal in any patient. There were no significant side-effects, particularly with respect to diabetic control, an important finding as lesions tended to relapse if treatment was stopped.