Is it Lucio Phenomenon or Necrotic Erythema Nodosum Leprosum?
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 boththe earlobes within a day, thus was referred to us.\nThere was no history of fever, arthralgia or any other constitutional symptoms in the past 2 days. He gave history of recurrent blistering of hands and feet for the past 1 year. On cutaneous examination, multiple hemorrhagic bullae measuring 1-2 cms were seen over the dorsum of both hands and fingers feet and toes [], with surrounding purpuric macules. Similar vesicles and erosions covered with hemorrhagic crust were present over the helices of both the ears []. Scattered angular purpuric macules were present over the extensor aspect of the arms and above the ankles. Within 2 days, these bullae had ruptured leaving large deep irregular ulcers with angulated margins.\nBesides this, there was diffuse infiltration of the face with saddle nose deformity. Skin over the back was diffusely infiltrated with a shiny appearance and intact sensation. The peripheral nerves were symmetrically thickened and non-tender. Bilateral glove and stocking anesthesia was present. Mobile ulnar claw deformity of both the hands was noted. He had resorption of the right little finger, pseudo-ainhum of the right fourth toe, and an amputated left second toe. Examination of the cranial nerves was normal in him.\nSlit skin smear from the earlobes revealed the presence of globi. The average bacteriological index was 4 +. With the above findings, a provisional diagnosis of diffuse non-nodular form of lepromatous leprosy with grade 2 deformity of hands and feet was made. A differential diagnosis of LP, drug-induced pseudoporphyria, drug-induced vasculitis, cryoglobulinemic vasculitis, and necrotic erythema nodosum leprosum were considered for the hemorrhagic bullae, ulcers, and purpuric macules.\nHemogram was normal, except for neutrophilic leukocytosis. Erythrocyte sedimentation rate was 56 mm in the first hour. Liver, renal function tests and urine examination were normal. VDRL (Venereal disease research laboratory test) for syphilis, ELISA for human immunodeficiency virus (HIV), and hepatitis B surface antigen were negative. Urine examination for porphyrins was negative. Serum cryoglobulins, rheumatoid factor (RF), and anti-nuclear antibody (ANA) were negative. Skiagram of both the hands and feet did not show any evidence of osteitis or osteomyelitis.\nBiopsy from the infiltrated skin over the back showed atrophic epidermis, free Grenz zone, diffuse and periadnexal macrophage granulomas with strong Fites' stain positivity for M. leprae consistent with lepromatous leprosy []. 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. We were not able to demonstrate the acid-fast bacilli within the endothelial cells.\nIn 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). There were no new lesions after start of therapy. Prednisolone was tapered after 1 month by 5 mg every 2 weeks and subsequently stopped. The ulcers healed slowly with depigmented scars after 10 weeks [].