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Radical chik: Chikungunya fever presenting as a toxic epidermal necrolysis-like eruption


DII small banner By Warren R. Heymann, MD
June 25, 2018

  Chikungunya-Fever.jpg
Chikungunya fever: morbilliform rash with islands of sparing. Photograph courtesy of Zawar Vijay, MD, Nashik, India.
Credit: JAAD
I have seen only one documented case of Chikungunya fever (CF), in a young woman who returned from Puerto Rico, presenting with high fever, joint pains, and a morbilliform rash (see image). Unfortunately, she will not be the last.

CF is caused by the Chikungunya virus, an RNA Flavivirus (which is related to the viruses responsible for Zika, dengue, yellow fever, and West Nile infections). Aedes aegypti and A. albopictus mosquitos are the main vectors. The emergence of CF is due to a multiplicity of factors, including climate change, urbanization, extensive agriculture, and global air transport. (1)
 
Riyaz reviewed 162 cases of CF (all but 5 confirmed by a positive IgM titer to the virus). Patients presented with either a single lesion or a combination of lesions. The most common presentation was an erythematous rash in 94 (58%) patients.  Most of the skin lesions developed during the acute phase of the disease, 2 to 3 days after the onset of fever (74.16%). Localized erythema and swelling of the pinnae mimicking erysipelas was observed in 7 patients. Similarly, erythema and edema of BCG scar and pre-existing post traumatic scars and striae resembling the ‘scar phenomenon’ of sarcoidosis were seen in 4 patients. Other lesions were vesicles and bullae over the extremities in 21 (12.96%).  Seven infants (4.32%), had an entirely different presentation with charring, vesicles and bullae, followed by peeling, clinically mimicking toxic epidermal necrolysis (TEN), but without mucosal involvement. Oral mucosal involvement was found in 22 (13.64%) patients in the form of multiple aphthae, erosions, and cheilitis. Bilateral involvement of conjunctiva was present in 14 (8.64%). Nasal erosions were seen in 8 (4.94%) patients. Two patients (1.24%) had scrotal ulcers. Mucosal lesions lasted for 7-10 days and subsided completely without any sequelae. The peculiar nail findings noted in our study were black lunulae, diffuse and longitudinal melanonychia, transverse pigmented bands in two patients each and a single case each of leukonychia and onychomadesis. Skin lesions subsided without any sequelae in the majority, except pigmentation in 62 patients (38.27%). Various types of pigmentation were observed, the most common site being the nose. (2) This is known as the “Chik sign”, which may endure for months (see DI&I April 24th, 2016).
 
Garg et al reported 21 children with CF (13 boys, 8 girls) presenting with vesico-bullous and necrotic lesions; purpura and necrosis was seen in 16 (76%) patients. Skin lesions resolved in 5-7 days, leaving behind hyperpigmentation in 7 (33.3%) patients and hypopigmentation in 3 (14.2%). Minor oral erosions were observed in 3 (14.2%) patients, and palmoplantar erythema was seen in 4 (19.04%). Importantly, most children were active, playful, and eating well through their illness (compared to patients with TEN). The authors concluded: “It is essential for dermatologists to understand the Stevens-Johnson syndrome and toxic epidermal necrolysis-like presentation of chikungunya and not to misinterpret it as true Stevens-Johnson syndrome and toxic epidermal necrolysis, which will lead to unnecessary intervention and management.” (3)
 
Although the pathomechanism for the epidermal necrosis is unknown, the presumption is that due to immunologic factors combined with a direct cytopathic effect of the virus. (2)

CF is usually self-limited, with the majority of symptoms resolving in 7 to 10 days. Rheumatologic, neurologic, cardiac, respiratory, renal, and ocular complications may ensue, lasting for months. Elderly patients are at risk for more severe disease. Hypertension, diabetes and cardiac comorbidities may contribute to a severe outcome of CF. (4)

Chikungunya means “that which bends up” in Bantu language of the Makonde people, due to the potentially devastating painful arthralgias accompanying CF. (5) Vector control is currently the only comprehensive solution for decreasing the number of CF cases. (1). Vaccines for CF are being developed, 2 of which have successfully completed phase I clinical trials. (6)

The late, iconic author Tom Wolfe coined the phrase “radical chic.” Hopefully a new vaccine will allow for a radical change in the incidence of this devastating disease.

Point to remember: CF may present mimic Stevens-Johnson and TEN, especially in children.

1. Paixão ES, et al. Zika, chikungunya and dengue: The causes and threats of new and re-emerging arboviral diseases. BMJ Glob Health 2017; 3: e000530.
2. Riyaz N, et al. Cutaneous manifestations of chikungunya during a recent epidemic in Calicut, north Kerala, south India. Indian J Dermatol Venereol Leprol 2010; 76: 671-6.
3. Garg T, et al. Stevens-Johnson syndrome and toxic epidermal necrolysis-like cutaneous presentation of chikungunya fever: A case series. Pediatr Dermatol 2018; 35: 392-6.
4. Badawi A, et al. Prevalence of chronic comorbidities in Chikungunya: A systematic review and meta-analysis. Int J Infect Dis 67; 2018: 107-113.
5. Bautista-Reyes E, et al. Chikungunya: Molecular aspects, clinical outcomes and pathogenesis. Rev Invest Clin 2017; 69: 299-307
6. Tharmarajah K, et al. Chikungunya: Vaccines and therapeutics. F1000Res 2017; 6: 2114.

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