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Monday, October 10, 2011

A case of lepromatous leprosy presenting as generalized cutis laxa

Authors: Rathnayake, GGDSK
Dissanayake, DMS
Karunasekera, P
Issue Date: 15-Sep-2007
Citation: Rathnayake, GGDSK; Dissanayake, DMS; Karunasekera, P. A case of lepromatous leprosy presenting as generalized cutis laxa. 27th Anniversary- Academic Sessions, PGIM Programme & Abstract Book. 2007; 50(1): 44-45
Language: en
Type: Article
Abstract: Leprosy is a common disease in the tropics. It has a diverse clinical presentation. Lepromatous leprosy is a systemic disease which is also a source of infection. Clinically, it presents with multiple symmetrical nodules, plaques, skin infiltration and can cause functional disability. It can also cause multi-organ involvement. Atypical presentation of leprosy has been reported causing diagnostic difficulty. Lepromatous leprosy still remains a major clinical challenge. Cutis laxa is a congenital or acquired disorder of connective tissues in which skin becomes inelastic and hangs loosely in folds. Histologically it shows elastolysis. The patients with cutis laxa appear prematurely aged and their faces resemble a bloodhound. Acquired cutis laxa has been reported following urticaria, angioedema, multiple myeloma, drug hyper-sensitivity, amyloidosis, sarcoidosis and extensive inflammatory conditions of the skin like SLE and RA. Localized elastolysis has been reported following leprosy. Case report We report a 50-year-old-man who had been previously healthy presenting with progressive generalized cutis laxa and blepharochalasis for 1 year associated with peripheral neuropathy. He also has axillary lymphadenopathy and purpura in both hands. No evidence of anaesthetic skin patches, inflammatory nodules, plaques, thickened peripheral nerves, pallor or hepatospleenomegaly was found. Granulomatous slack skin syndrome, multiple myeloma or atypical presentation of leprosy were clinically suspected.Skin biopsy showed evidence oflepromatous leprosy and stained positive for acid fast bacilli. Slit skin smear shows bacillary index of 4+. Lymph node biopsy showed features of leprosy, but no evidence of malignancy. Screening for multiple myeloma was negative. Lepromatous leprosy was diagnosed and multibacillary anti leprosy treatment commenced. We report this case to emphasize that leprosy can present in atypical forms making clinical diagnosis difficult. Inflammatory cells and mediators are thought to play a role in pathogenesis of cutis laxa following inflammatory skin conditions.