A case of malignant atrophic papulosis successfully treated with nicotine patches
The following is an extract from the British Journal of Dermatology 2003:
SIR, Malignant atrophic papulosis (Degos' disease) is a rare disorder of unknown cause, which is characterized by thrombotic vasculopathy affecting the skin, gastrointestinal tract, central nervous system, and occasionally other organs. It is often lethal because of gastrointestinal involvement. The cutaneous lesions consist of characteristic papules with porcelain-white central atrophy and telangiectatic borders. Similar lesions are seen on the serosal surface of the gastrointestinal tract; perforation of the tract is the most frequent cause of death. Treatments that have been tried for this disorder include antiplatelet therapy (aspirin and dipyridamole), 1,2 fibrinolytic therapy (phenformin and ethyloestrenol), 3 anisoylated plasminogen streptokinase activator and urokinase. 4 However, there is no really satisfactory treatment. 4 We describe a woman with this disorder whose skin lesions and gastrointestinal symptoms were treated successfully with nicotine patches.
A 24-year-old woman with multiple skin lesions was referred to our department. She first noticed the skin lesions on her lower extremities 4 months prior to her first visit. Examination revealed multiple papules or macules 4–8 mm in diameter with slightly depressed, porcelain-white, atrophic centres surrounded by telangiectatic rims, which were disseminated predominantly on her legs and trunk (Fig. 1a). Skin biopsy of the left thigh revealed a clearly demarcated necrotic and atrophic epidermis, and lymphocytes and neutrophils infiltrating in the vicinity of vessels in the dermis. Arterioles occluded by thrombi in the mid-dermis were seen in a serial section (Fig. 2). Results of haematological, biochemical and immunological tests were all within normal limits. Antinuclear antibody was negative. Antibodies for double-stranded DNA, Sm and phospholipids were not detected. Based on the results of clinical, histopathological and laboratory examinations, a diagnosis of malignant atrophic papulosis was made. Stool tests for occult blood were negative on three consecutive days. Endoscopic examination, the upper gastrointestinal series and an abdominal echogram revealed no abnormalities in the tract, although she had had recurrent episodes of nausea and abdominal pain for 3 months. We started transdermal nicotine patches without any other systemic or topical treatments. She applied a patch that released 5 mg of nicotine to her thigh once daily and wore it for 24 h. In 2 weeks, the treatment resulted in cessation of new lesion development and gastrointestinal disturbances. However, new lesions started to appear 3 weeks after withdrawal of the nicotine patches. When the patches were reintroduced, the lesions improved again (Fig. 1b). Her disease has been under control with the nicotine patches for 2 years. She had no side-effects such as skin irritation, sleep disturbance, dyspepsia, or change in blood pressure.
We demonstrated previously that nicotine increases peripheral cutaneous blood flow 5 and is beneficial for the treatment of skin disorders due to circulation disturbances, including pyoderma gangrenosum, 6 digital ulceration in Buerger's disease 7 and orogenital ulceration in Behc¸et's disease. 8 Scheid et al . also reported a patient with aphthous ulcers due to Behc¸et's syndrome who benefited from nicotine patches. 9 Based on these results, we tried nicotine patches in the present patient because malignant atrophic papulosis is characterized by endovasculitis and subsequent circulation disturbances of the skin and gastrointestinal tract. Classically, this disorder has been considered to be fatal mainly because of intestinal perforation. 1 A study by Su et al . demonstrated that patients with typical cutaneous lesions who lacked systemic involvement showed a benign clinical course, 10 whereas Degos reported that cutaneous lesions usually precede intestinal involvement and that it appeared likely that most cases reported as having only cutaneous lesions developed systemic lesions sooner or later in view of the long interval between cutaneous and systemic manifestations. 1
While we have already demonstrated the beneficial effects of nicotine on pyoderma gangrenosum, 6 digital ulceration in Buerger's disease 7 and orogenital ulceration in Behc¸et's disease, 8 the outcome of the present study may be very important because nicotine may be effective in treating systemic disorders affecting the vasculature and may be used safely for long-term treatment.
T.Kanekura
Y.Uchino
T.Kanzaki
Department of Dermatology,
Kagoshima University Faculty of Medicine, 8-35-1 Sakuragaoka, Kagoshima 890–8520, Japan
E-mail: takurok@m2.kagoshima-u.ac.jp
References
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