Extracutaneous manifestations and long-term sequelae of Stevens-Johnson syndrome/toxic epidermal necrolysis

Vercueil, André; Walsh, Sarah

doi:10.1111/bjd.13631

Cited by 3 publications

(2 citation statements)

References 3 publications

(4 reference statements)

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“…M. pneumoniae is an obligate intercellular organism associated with pneumonia in pediatric and young adult populations [ 1 ]. In addition to respiratory involvement 7%-8% of cases result in extrapulmonary manifestation, including arthritis (<3%), hepatitis (<5%), pericarditis (<1%), hemolytic anemia (<5%), aseptic meningitis (<1%), and SJS (<1%) [ 6 ]. M. pneumoniae -associated SJS (atypical SJS) is clinically diagnosed based on the distribution of the rash, along with associated clinical findings [ 4 ].…”

Section: Introductionmentioning

confidence: 99%

Atypical Stevens-Johnson Syndrome Associated With Mycoplasma Pneumoniae

Beheshti¹,

Cusack²

2022

Cureus

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Mycoplasma pneumoniae primarily causes atypical pneumonia in children and young adults. 7%-8% of patients with M. pneumoniae infections may experience extra-pulmonary manifestations, including M. pneumoniae-associated Stevens-Johnson Syndrome (SJS), also known as atypical SJS. In recent literature, there have been a few reports of isolated mucositis in children with M. pneumoniae infections. Due to significant overlap with several diseases, including autoimmune disease and infections, atypical mucositis associated with M. pneumoniae is often a diagnostic challenge. In addition, due to limited cases of M. pneumoniae-associated SJS, there is no established standardized treatment guideline that has been shown to reduce hospitalization duration and/or disease progression associated with M. pneumoniae-associated SJS. We report a case of isolated mucositis in the absence of cutaneous involvement in a 10-year-old patient with an acute M. pneumoniae infection. Examination revealed erythematous ulcerations of his lips and pharynx with patchy exudates and bilateral submandibular lymphadenopathy. Laboratory investigation revealed a negative respiratory polymerase chain reaction (PCR) panel, which included M. pneumoniae. Further testing revealed a positive M. pneumoniae immunoglobulin M (IgM) titer on enzyme immunoassay. The diagnosis of atypical SJS was made secondary to M. pneumoniae. Treatment was initiated with systemic steroids and oral antibiotics. Limitations in diagnostic testing for M. pneumoniae in combination with non-specific clinical presentation make for challenges in confirming this pattern of SJS due to a primary M. pneumoniae infection. In this case, serological testing confirmed our suspected diagnosis, which guided treatment and helped reveal some of the difficulties in diagnosing and managing M. pneumoniae-associated SJS.

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Section: Introductionmentioning

confidence: 99%

Atypical Stevens-Johnson Syndrome Associated With Mycoplasma Pneumoniae

Beheshti¹,

Cusack²

2022

Cureus

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“…However, advances in genetics have revealed interactions of specific HLA alleles with EN‐associated drugs, giving more insight in EN pathogenesis 8,9 . As survivors of EN suffer from a variety of sequelae affecting for instance the eyes and respiration, a disregarded chronic phase of the illness has recently been highlighted 10,11 …”

Section: Introductionmentioning

confidence: 99%

Clinical and pathogenic aspects of the severe cutaneous adverse reaction epidermal necrolysis (EN)

Kuijper

French

Tensen

et al. 2020

Acad Dermatol Venereol

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The severe cutaneous adverse reaction epidermal necrolysis (EN) which includes toxic epidermal necrolysis and the milder Stevens‐Johnson syndrome is characterized by epidermal loss due to massive keratinocyte apoptosis and/or necroptosis. EN is often caused by a drug mediating a specific TCR‐HLA interaction via the (pro)hapten, pharmacological interaction or altered peptide loading mechanism involving a self‐peptide presented by keratinocytes. (Memory) CD8 + T cells are activated and exhibit cytotoxicity against keratinocytes via the perforin/granzyme B and granulysin pathway and Fas/FasL interaction. Alternatively drug‐induced annexin release by CD14 + monocytes can induce formyl peptide receptor 1 death of keratinocytes by necroptosis. Subsequent keratinocyte death stimulates local inflammation, activating other immune cells producing pro‐inflammatory molecules and downregulating regulatory T cells. Widespread epidermal necrolysis and inflammation can induce life‐threatening systemic effects, leading to high mortality rates. Research into genetic susceptibility aims to identify risk factors for eventual prevention of EN. Specific HLA class I alleles show the strongest association with EN, but risk variants have also been identified in genes involved in drug metabolism, cellular drug uptake, peptide presentation and function of CD8 + T cells and other immune cells involved in cytotoxic responses. After the acute phase of EN, long‐term symptoms can remain or arise mainly affecting the skin and eyes. Mucosal sequelae are characterized by occlusions and strictures due to adherence of denuded surfaces and fibrosis following mucosal inflammation. In addition, systemic pathology can cause acute and chronic hepatic and renal symptoms. EN has a large psychological impact and strongly affects health‐related quality of life among EN survivors.

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Stevens-Johnson syndrome and toxic epidermal necrolysis: case series and brief literature review

Aloyan,

Kalikyan,

Harutyunyan

et al. 2023

AJHMS

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Stevens-Johnson syndrome (SJS) and toxic epi- dermal necrolysis (TEN) are rare forms of delayed-type drug hypersensitivity reactions (DHRs) with extensive skin necrosis and exfoliation. Drugs are the main cause of SJS and TEN in both adults and children. The most common medications are allopurinol, aromatic antiepileptics and sulfonamides with seasonal, geographical and ethnic variations. The purpose of this paper is to present the cases of SJS/TEN which were successfully treated during the year of 2021 in our department and to provide a brief literature review.

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Extracutaneous manifestations and long-term sequelae of Stevens-Johnson syndrome/toxic epidermal necrolysis

Cited by 3 publications

References 3 publications

Atypical Stevens-Johnson Syndrome Associated With Mycoplasma Pneumoniae

Atypical Stevens-Johnson Syndrome Associated With Mycoplasma Pneumoniae

Clinical and pathogenic aspects of the severe cutaneous adverse reaction epidermal necrolysis (EN)

Stevens-Johnson syndrome and toxic epidermal necrolysis: case series and brief literature review

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