Skip to main content
Top
Published in: BMC Dermatology 1/2017

Open Access 01-12-2017 | Case report

A case report of erythroderma in a patient with borderline leprosy on reversal reaction: a result of the exacerbated reaction?

Authors: Denis Miyashiro, Ana Paula Vieira, Maria Angela Bianconcini Trindade, João Avancini, José Antonio Sanches, Gil Benard

Published in: BMC Dermatology | Issue 1/2017

Login to get access

Abstract

Background

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.

Case presentation

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.

Conclusions

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.
Appendix
Available only for authorised users
Literature
1.
go back to reference Sehgal VN, Srivastava G, Sardana K. Erythroderma/exfoliative dermatitis: a synopsis. Int J Dermatol. 2004;43(1):39–47.CrossRefPubMed Sehgal VN, Srivastava G, Sardana K. Erythroderma/exfoliative dermatitis: a synopsis. Int J Dermatol. 2004;43(1):39–47.CrossRefPubMed
3.
go back to reference Prashar A, Narang T, Saikia UN, Dogra S. Deck chair sign in lepromatous leprosy. Lepr Rev. 2013;84(3):252–4.PubMed Prashar A, Narang T, Saikia UN, Dogra S. Deck chair sign in lepromatous leprosy. Lepr Rev. 2013;84(3):252–4.PubMed
5.
go back to reference 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.CrossRefPubMedPubMedCentral 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.CrossRefPubMedPubMedCentral
6.
go back to reference 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.CrossRefPubMedPubMedCentral 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.CrossRefPubMedPubMedCentral
7.
go back to reference Walker SL, Lockwood DN. Leprosy type 1 (reversal) reactions and their management. Lepr Rev. 2008;79(4):372–86.PubMed Walker SL, Lockwood DN. Leprosy type 1 (reversal) reactions and their management. Lepr Rev. 2008;79(4):372–86.PubMed
8.
go back to reference 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.CrossRefPubMedPubMedCentral 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.CrossRefPubMedPubMedCentral
9.
go back to reference 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.PubMed 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.PubMed
10.
go back to reference 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.CrossRefPubMed 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.CrossRefPubMed
11.
go back to reference 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.CrossRefPubMed 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.CrossRefPubMed
12.
go back to reference 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.CrossRefPubMedPubMedCentral 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.CrossRefPubMedPubMedCentral
13.
go back to reference 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.CrossRefPubMedPubMedCentral 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.CrossRefPubMedPubMedCentral
14.
go back to reference Romagnani S. T-cell subsets (Th1 versus Th2). Ann Allergy Asthma Immunol. 2000;85(1):9–18. quiz, 21CrossRefPubMed Romagnani S. T-cell subsets (Th1 versus Th2). Ann Allergy Asthma Immunol. 2000;85(1):9–18. quiz, 21CrossRefPubMed
15.
go back to reference Noack M, Miossec P. Th17 and regulatory T cell balance in autoimmune and inflammatory diseases. Autoimmun Rev. 2014;13(6):668–77.CrossRefPubMed Noack M, Miossec P. Th17 and regulatory T cell balance in autoimmune and inflammatory diseases. Autoimmun Rev. 2014;13(6):668–77.CrossRefPubMed
Metadata
Title
A case report of erythroderma in a patient with borderline leprosy on reversal reaction: a result of the exacerbated reaction?
Authors
Denis Miyashiro
Ana Paula Vieira
Maria Angela Bianconcini Trindade
João Avancini
José Antonio Sanches
Gil Benard
Publication date
01-12-2017
Publisher
BioMed Central
Published in
BMC Dermatology / Issue 1/2017
Electronic ISSN: 1471-5945
DOI
https://doi.org/10.1186/s12895-017-0068-3

Other articles of this Issue 1/2017

BMC Dermatology 1/2017 Go to the issue