[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.

Effect of spinal cord stimulation in Type I complex regional pain syndrome with 2 rare severe cutaneous manifestations.

The authors present the case of a 49-year-old female patient with complex regional pain syndrome-Type I (CRPSI) who was suffering from nonhealing wounds and giant bullae, which dramatically improved after spinal cord stimulation (SCS). The scientific literature concerning severe cutaneous manifestations of CRPS-I and their treatment is reviewed. Nonhealing wounds and bullae are rare manifestations of CRPS-I that are extremely difficult to treat. Immediate improvement of both wounds and bullae after SCS, such as in this case, has not been reported previously in literature. Considering the rapidly progressive nature of these severe skin manifestations, immediate treatment, possibly with SCS, is mandatory.

Narrowband UVB therapy as an effective treatment for Schamberg's disease.

A 33-year-old man presented with a 3-month history of a widespread pigmented purpuric eruption over his trunk and limbs. The clinical presentation and histology were consistent with a diagnosis of Schamberg's disease. The rash initially cleared following a short course of oral prednisolone at 25 mg daily for 3 weeks, which was weaned over the subsequent 4 weeks. Topical mometasone furoate ointment 0.1% daily was also applied to active areas. The rash recurred when prednisolone was reduced to below 5 mg per day. To prevent a further recurrence with weaning prednisolone, narrowband UVB therapy was commenced three times per week. The patient was continued on UV therapy over the next 5 months. The rash would flare after 2 to 3 weeks without treatment. The frequency of UV therapy was weaned and the patient remained clear on as little as one treatment every 2 weeks. Any further reduction, however, was associated with a recurrence. Narrowband UVB therapy should be considered for difficult or persistent cases of pigmented purpuric eruption.

Successful treatment of lichen amyloidosis with combined bath PUVA photochemotherapy and oral acitretin.

Lichen amyloidosis (LA) is a chronic, pruritic skin disorder characterized by brownish-grey papules on extensor surfaces of legs and rarely on the trunk. Thioflavin T-positive amyloid deposits are found in the papillary dermis of affected skin, which is the only organ involved. A variety of therapeutic regimens for lichen amyloidosis have been described; however, in many cases with only limited effect. We report on two patients with lichen amyloidosis with typical clinical symptoms not responding to local treatment. A combined regimen with bath psoralen ultraviolet A (PUVA) and oral acitretin was initiated, resulting in nearly complete resolution of the papules and impressive relief from the severe pruritus. The beneficial response has persisted for 8 months. The suggested combined therapy with bath PUVA photochemotherapy and oral acitretin represents an efficacious and practical treatment modality for lichen amyloidosis with long-lasting effects.

Livedoid vasculopathy: long-term follow-up results following hyperbaric oxygen therapy.

BACKGROUND: Livedoid vasculopathy, also known as atrophie blanche, is a recurrent painful vasculopathy appearing mostly on the lower limbs. Treatment is challenging and relapses are frequent. OBJECTIVES: To analyse the long-term effect and safety of hyperbaric oxygen (HBO) therapy in treating livedoid vasculopathy. METHODS: Twelve patients with active livedoid vasculopathy were included in this study. All patients underwent HBO therapy five times a week. Each week photographs were taken and the total dose of analgesics was recorded. Side-effects were documented and assessed. Recurrence was defined as the presence of skin ulceration. RESULTS: Of the eight patients who completed the treatment, resumption of ambulation and reduction of analgesics were achieved at an average of 4.9 HBO therapy sessions. Leg ulcers in all eight patients healed completely at a mean of 3.4 weeks (range 2-5 weeks). Six patients suffered relapses of ulceration and responded to additional HBO therapy. No significant side-effects were found. CONCLUSIONS: HBO is a relatively safe, fast and effective method to treat patients with livedoid vasculopathy.