[Late-occurring cutaneous vasculitis after successful treatment of diffuse lepromatous leprosy: Lucio's phenomenon]. 2007

S Fenniche, and R Benmously, and M Sfia, and L Daoud, and A Debbiche, and M Ben Ayed, and I Mokhtar
Service de dermatologie, HĂ´pital Habib Thameur, 1008 Tunis, Tunisie. fenniche.samy@wanadoo.tn

O Lucio's phenomenon is an uncommon type 2 reactional state occurring exclusively in patients with diffuse lepromatous leprosy (Lucio-Latapi leprosy). Previous case reports have been most frequent in Central America and rare in Asia and Africa. Lucio's phenomenon is characterized by necrotic ulcerations of the skin preferentially on the lower extremities usually in association with ongoing Lucio lepromatosis. The purpose of this report is to describe an unusual case of Lucio's phenomenon occurring four years after successful treatment of diffuse lepromatous leprosy. The patient was a 51-year-old man who had presented diffuse lepromatous leprosy ongoing since 1998. Diagnosis was documented based on histological and bacteriologic evidence. After successful treatment using dapsone (100 mg/d), rifadine (600 mg/month) and ethionamide (250 mg/d), the patient was lost from follow-up for 4 years. In January 2005, he consulted again for alteration of general status. Clinical examination showed inflammatory livedo on the lower extremities in association with several infiltrating maculo-papular lesions and painful erythemato-pupuric lesions on the legs and buttocks. The patient's skin was dry, shiny and galabrous with alopecia of the eyelashes and eyebrows. Examination of smear samples (skin and nasal) to identify mycobacterium leprae was negative. Histological study demonstrated epidermic necrosis with aspects of leucocytoclastic vasculitis. No Virchow cells were detected and Ziehl staining was negative. Search for circulating immune complexes and antiphospholipid antibodies was negative. Diagnosis of Lucio's phenomenon was made and the patient was treated using prednisone at a dose of 1 mg/kg/d in association with rifampicine (600 mg/month) and dapsone (100 mg/d). Outcome was favorable after one month of treatment. Lucio's phenomenon has rarely been observed in Tunisia. To our knowledge this is the third case reported from Tunisia and only 13 cases have been reported in the world since 1983. In all cases including the two from Tunisia, Lucio's phenomenon occurred during the course of treatment of ongoing Lucio-Latapi lepromatous leprosy (2). The remarkable features of our case are that Lucio's phenomenon occurred a long time after successful treatment of lepromatous leprosy and that the patient responded promptly to treatment. The pathogenesis of Lucio's phenomenon is often compared with that of erythema nodosum leprosum. Discussion focuses on pathophysiologic features and natural course of Lucio's phenomenon.

UI MeSH Term Description Entries
D007917 Leprostatic Agents Substances that suppress Mycobacterium leprae, ameliorate the clinical manifestations of leprosy, and/or reduce the incidence and severity of leprous reactions. Antileprotic Agents,Leprostatics,Agents, Antileprotic,Agents, Leprostatic
D008297 Male Males
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D011241 Prednisone A synthetic anti-inflammatory glucocorticoid derived from CORTISONE. It is biologically inert and converted to PREDNISOLONE in the liver. Dehydrocortisone,delta-Cortisone,Apo-Prednisone,Cortan,Cortancyl,Cutason,Dacortin,Decortin,Decortisyl,Deltasone,Encorton,Encortone,Enkortolon,Kortancyl,Liquid Pred,Meticorten,Orasone,Panafcort,Panasol,Predni Tablinen,Prednidib,Predniment,Prednison Acsis,Prednison Galen,Prednison Hexal,Pronisone,Rectodelt,Sone,Sterapred,Ultracorten,Winpred,Acsis, Prednison
D005938 Glucocorticoids A group of CORTICOSTEROIDS that affect carbohydrate metabolism (GLUCONEOGENESIS, liver glycogen deposition, elevation of BLOOD SUGAR), inhibit ADRENOCORTICOTROPIC HORMONE secretion, and possess pronounced anti-inflammatory activity. They also play a role in fat and protein metabolism, maintenance of arterial blood pressure, alteration of the connective tissue response to injury, reduction in the number of circulating lymphocytes, and functioning of the central nervous system. Glucocorticoid,Glucocorticoid Effect,Glucorticoid Effects,Effect, Glucocorticoid,Effects, Glucorticoid
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D014657 Vasculitis Inflammation of any one of the blood vessels, including the ARTERIES; VEINS; and rest of the vasculature system in the body. Angiitis,Angiitides,Vasculitides
D015440 Leprosy, Lepromatous A chronic communicable infection which is a principal or polar form of LEPROSY. This disorder is caused by MYCOBACTERIUM LEPRAE and produces diffuse granulomatous skin lesions in the form of nodules, macules, or papules. The peripheral nerves are involved symmetrically and neural sequelae occur in the advanced stage. Leprosy, Cutaneous,Leprosy, Nodular,Cutaneous Leprosies,Cutaneous Leprosy,Lepromatous Leprosies,Lepromatous Leprosy,Leprosies, Cutaneous,Leprosies, Lepromatous,Leprosies, Nodular,Nodular Leprosies,Nodular Leprosy
D017445 Skin Diseases, Vascular Skin diseases affecting or involving the cutaneous blood vessels and generally manifested as inflammation, swelling, erythema, or necrosis in the affected area. Cutaneous Vasculitis,Vascular Skin Diseases,Cutaneous Vasculitides,Skin Disease, Vascular,Vascular Skin Disease,Vasculitis, Cutaneous

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