Reactive perforating leprosy, erythema multiforme-like reactions, sweet's syndrome-like reactions as atypical clinical manifestations of Type 2 leprosy reaction

Gunawan, Hendra; Yogya, Yuri; Hafinah, Risty; Marsella, Rachel; Ermawaty, Devi; Suwarsa, Oki

doi:10.4103/ijmy.ijmy_186_17

Cited by 13 publications

(7 citation statements)

References 2 publications

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“…Atypical ENL lesions were predominant in male patients with an approximate male to female ratio of 2.7 : 1 and an age range of 10–73 years with a mean ± standard deviation (SD) of 41.5 ± 44.5. A list of reported cases of atypical ENL is demonstrated in Table 1 [4–59].…”

Section: Resultsmentioning

confidence: 99%

Atypical presentations of erythema nodosum leprosum: Diagnostic and therapeutic aspects

Alakad

Nofal

Assaf

2021

J Deutsche Derma Gesell

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Summary Classic erythema nodosum leprosum (ENL) is characterized clinically by abrupt eruption of tender erythematous nodules, papules and plaques. Variable atypical patterns have been described, for example pustular, bullous, ulcerative, necrotic and Sweet’s syndrome‐like ENL. We aim to review previously reported cases of atypical ENL addressing the diagnostic and therapeutic aspects of these uncommon presentations. A search of medical literature for all cases of atypical ENL was conducted in the PubMed database till 2020. Data of patients with atypical ENL were collected and analyzed to describe the epidemiological, clinico‐histological and therapeutic features. The major five clinically described presentations of atypical ENL include vesiculo‐bullous lesions (46 % of patients), ulcero‐necrotic lesions (41 %), erythema multiforme‐like lesions (28 %), Sweet’s syndrome‐like lesions (11 %) and pustules (9 %). The skin lesions were accompanied by fever and constitutional symptoms in all patients. Oral steroids and thalidomide were the main lines of therapy in most of the reported patients. Dermatologists and pathologists should keep in mind the clinical variability of ENL to avoid misdiagnosis and delayed management. Early recognition can help control disease progression and save the patients from further complications.

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Section: Resultsmentioning

confidence: 99%

Atypical presentations of erythema nodosum leprosum: Diagnostic and therapeutic aspects

Alakad

Nofal

Assaf

2021

J Deutsche Derma Gesell

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“…ENL is a type III hypersensitivity reaction and many of its atypical variants have been described including pustular, bullous, erythema multiforme-like and SS-like. [12][13][14][15][16] The overall prevalence of type 2 lepra reaction is estimated at 4.8%, with 0.3% having pustulo-necrotic ENL. 17 Previous studies have reported 48 and 11 patients out of 292 with ulcerated and necrotic ENL respectively.…”

Section: Discussionmentioning

confidence: 99%

Unusual non-trophic ulcerations in leprosy: A case series of 17 patients

et al. 2021

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Non-neuropathic ulcers in leprosy patients are infrequently seen, and atypical presentations are prone to misdiagnosis. We evaluated diagnosed cases of leprosy between January 2017 and January 2020 for the presence of cutaneous ulceration, Ridley–Jopling subtype of leprosy, reactions and histologic features of these ulcerations. Treatment was given as WHO recommended multi-bacillary multi-drug therapy. We found 17/386 leprosy patients with non-neuropathic ulcers. We describe three causes – spontaneous cutaneous ulceration in lepromatous leprosy (one nodular and one diffuse), lepra reactions (five patients with type 1; nine with type 2, further categorised into ulcerated Sweet syndrome-like who also had pseudoepitheliomatous hyperplasia, pustulo-necrotic and necrotic erythema nodosum leprosum) and Lucio phenomenon (one patient). Our series draws attention towards the different faces of non-neuropathic ulcers in leprosy, including some atypical and novel presentations.

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“…Besides nodular lesions, various reported atypical presentations of ENL include pustular, bullous, ulcerated, livedo reticularis, 3 and erythema multiforme-like lesions. 4,5 A Sweet-like syndrome has been reported 6,7 pityriasis lichenoides et varioliformis acuta (PLEVA) like ENL 8 reactive perforating 9 and Lucio phenomenon.…”

Section: Discussionmentioning

confidence: 99%

An uncommon variant of erythema nodosum leprosum responding well to methotrexate: Report of two cases

et al. 2021

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Reactive perforating leprosy, erythema multiforme-like reactions, sweet's syndrome-like reactions as atypical clinical manifestations of Type 2 leprosy reaction

Cited by 13 publications

References 2 publications

Atypical presentations of erythema nodosum leprosum: Diagnostic and therapeutic aspects

Atypical presentations of erythema nodosum leprosum: Diagnostic and therapeutic aspects

Unusual non-trophic ulcerations in leprosy: A case series of 17 patients

An uncommon variant of erythema nodosum leprosum responding well to methotrexate: Report of two cases

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