Is it lucio phenomenon or necrotic erythema nodosum leprosum?

Abstract

Lucio phenomenon (LP) or erythema necroticans is a relatively rare, peculiar reaction pattern occurring in untreated lepromatous (LL) or borderline lepromatous (BL) leprosy cases. A 38-year-old male, a cook by occupation, was referred to the dermatology clinic from otolaryngology department with blistering over both the hands and feet of 2 days duration. He had been admitted 1 week back with epistaxis and nasopharyngeal myiasis in otolaryngology department. He was started on systemic antibiotics gentamycin, crystalline penicillin, and metronidazole with nasal instillation of turpentine oil 2 drops 6 times a day. Two days later, he had developed edema with painless hemorrhagic blistering over the dorsum of left hand followed by involvement of the right hand, dorsa of both feet, and both the earlobes within a day. Histopathology of the blister showed sub-epidermal blister, with necrotizing leukocytoclastic vasculitis of papillary dermal vessels with thrombosis, numerous acid-fast bacilli in macrophages, and macrophage granulomas extending up to subcutis. In view of the absent fever or constitutional symptoms, and the classical angular infarcts and hemorrhagic blisters evolving into ulcers with angulated margins, we considered LP as the most likely diagnosis. The patient was started on a combination of WHO recommended multibacillary anti-leprosy therapy and prednisolone (40 mg/day).

Lucio’s phenomenon: A report of two cases and review of the literature

“…Case 2 had received treatment for Lepromatous Leprosy 5 years prior to admission. [8] Both patients developed a necrotizing vasculitis reported as Lucio's phenomenon, one of the clinical and pathological forms of Lepromatous Leprosy, which has been reported to occur in patients with chronic untreated LL 1 to 3 years after disease onset and is endemic to Central America and Mexico. [4,9] Clinical manifestations of Lucio's phenomenon consist of a variety of skin lesions from painful ecchymosis or macular purpura to blisters that rupture and develop ulcers.…”

“…Lucio's phenomenon was first described by Ladislao de la Pascua (1844) and was originally described under the name of "Greek Elephantiasis", as a severe necrotizing vasculitic reaction characterized by the appearance of ulcerative lesions that affect the extremities of patients with LL or BL typically 1 to 3 years after disease onset without adequate treatment. [2,[5][6][7][8][9] In this article we present 2 cases of Lucio's phenomenon, a complex and uncommon condition which is difficult to diagnose and must be identified promptly in order to prevent the adverse outcomes associated with it.…”

Lucio’s phenomenon, an uncommon occurrence among leprosy patients in Sri Lanka

“…It can also be the preliminary symptom introducing the lepromatous version of the disease. Rarely, it can present in an already fully treated diffuse lepromatous leprosy patient in the absence of bacilli, even years later [ 4 , 5 , 15 , 16 ].…”

“…Lucio’s phenomenon is one such manifestation. First described in detail by Lucio and Alvarado and further elaborated by Latapi and Franken nearly a century later, Lucio’s phenomenon is a rare form of reaction seen in pure lepromatous leprosy (LL) or borderline lepromatous leprosy (BL) [ 2 – 4 ]. Some consider this entity a separate form of leprosy and define it as Lucio’s leprosy [ 5 , 6 ] and attribute it to a newly discovered species, M. lepromatosis [ 7 , 8 ].…”

Diffuse Multibacillary Leprosy of Lucio and Latapí with Lucio’s Phenomenon, Peru

“…In addition, the patient’s condition met the 3 criteria that define Lucio’s phenomenon, according to the international literature: skin ulceration, vascular thrombosis, and invasion of blood vessels by leprosy bacilli ( 4 , 6 , 9 ). The term Lucio’s phenomenon should only be used when there is correlation of clinical and anatomic findings and in accordance with strict clinical criteria ( 4 ) The physiopathological mechanism of Lucio’s phenomenon requires further study to be properly understood ( 7 ).…”