A case report of erythroderma in a patient with borderline leprosy on reversal reaction: a result of the exacerbated reaction?
Tóm tắt
Erythroderma is characterized by erythema and scaling affecting more than 90% of the body surface area. Inflammatory, neoplastic and, more rarely, infectious diseases may culminate with erythroderma. Diagnosis of the underlying disorder is therefore crucial to institute the appropriate therapy. Leprosy is a chronic infectious disease that is endemic in Brazil. Here we present an unusual case of leprosy and reversal reaction causing erythroderma, and we discuss the underlying immunological mechanisms which could contribute to the generalized skin inflammation. We report a case of a patient with reversal reaction (RR) in borderline borderline leprosy presenting with erythroderma and neural disabilities. Histopathology of the skin showed regular acanthosis and spongiosis in the epidermis and, in the dermis, compact epithelioid granulomas as well as grouped and isolated bacilli. This duality probably reflects the transition from an anergic/multibacillary state to a state of more effective immunity and bacillary control, typical of RR. Leprosy was successfully treated with WHO’s multidrug therapy, plus prednisone for controlling the RR; the erythroderma resolved in parallel with this treatment. Immunologic studies showed in situ predominance of IFNγ + over IL-4+ lymphocytes and of IL-17+ over Foxp3+ lymphocytes, suggesting an exacerbated Th-1/Th-17 immunoreactivity and poor Th-2 and regulatory T-cell responses. Circulating Tregs were also diminished. We hypothesize that the flare-up of anti-mycobacteria immunoreactivity that underlies RR may have triggered the intense inflammatory skin lesions that culminated with erythroderma. This case report highlights the importance of thorough clinical examination of erythrodermic patients in search for its etiology and suggests that an intense and probably uncontrolled leprosy RR can culminate in the development of erythroderma.
Tài liệu tham khảo
Sehgal VN, Srivastava G, Sardana K. Erythroderma/exfoliative dermatitis: a synopsis. Int J Dermatol. 2004;43(1):39–47.
Shenoy MM, Bendigeri MA, Kamath PR, Vishal B. Diffuse leprosy with “deck-chair” sign. Indian Dermatol Online J. 2015;6(3):204–6.
Prashar A, Narang T, Saikia UN, Dogra S. Deck chair sign in lepromatous leprosy. Lepr Rev. 2013;84(3):252–4.
White C, Franco-Paredes C. Leprosy in the 21st century. Clin Microbiol Rev. 2015;28(1):80–94.
Palermo ML, Pagliari C, Trindade MA, Yamashitafuji TM, Duarte AJ, Cacere CR, et al. Increased expression of regulatory T cells and down-regulatory molecules in lepromatous leprosy. Am J Trop Med Hyg. 2012;86(5):878–83.
Vieira AP, Trindade MA, Pagliari C, Avancini J, Sakai-Valente NY, Duarte AJ, et al. Development of type 2, but not type 1, leprosy reactions is associated with a severe reduction of circulating and in situ regulatory T-cells. Am J Trop Med Hyg. 2016;94(4):721–7.
Walker SL, Lockwood DN. Leprosy type 1 (reversal) reactions and their management. Lepr Rev. 2008;79(4):372–86.
Fonseca AB, Simon MD, Cazzaniga RA, de Moura TR, de Almeida RP, Duthie MS, et al. The influence of innate and adaptative immune responses on the differential clinical outcomes of leprosy. Infect Dis Poverty. 2017;6(1):5.
Verhagen CE, Wierenga EA, Buffing AA, Chand MA, Faber WR, Das PK. Reversal reaction in borderline leprosy is associated with a polarized shift to type 1-like mycobacterium leprae T cell reactivity in lesional skin: a follow-up study. J Immunol. 1997;159(9):4474–83.
Sigurdsson V, Toonstra J, Bihari IC, Bruijnzeel-Koomen CA, van Vloten WA, Thepen T. Interleukin 4 and interferon-gamma expression of the dermal infiltrate in patients with erythroderma and mycosis fungoides. An immuno-histochemical study. J Cutan Pathol. 2000;27(9):429–35.
Moy AP, Murali M, Kroshinsky D, Duncan LM, Nazarian RM. Immunologic overlap of helper T-cell subtypes 17 and 22 in Erythrodermic psoriasis and atopic dermatitis. JAMA Dermatol. 2015;151(7):753–60.
Sadhu S, Khaitan BK, Joshi B, Sengupta U, Nautiyal AK, Mitra DK. Reciprocity between regulatory T cells and Th17 cells: relevance to polarized immunity in leprosy. PLoS Negl Trop Dis. 2016;10(1):e0004338.
Saini C, Siddiqui A, Ramesh V, Nath I. Leprosy reactions show increased Th17 cell activity and reduced FOXP3+ Tregs with concomitant decrease in TGF-beta and Increase in IL-6. PLoS Negl Trop Dis. 2016;10(4):e0004592.
Romagnani S. T-cell subsets (Th1 versus Th2). Ann Allergy Asthma Immunol. 2000;85(1):9–18. quiz, 21
Noack M, Miossec P. Th17 and regulatory T cell balance in autoimmune and inflammatory diseases. Autoimmun Rev. 2014;13(6):668–77.