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Pirfenidone‐induced severe phototoxic reaction in a patient with idiopathic lung fibrosis
Author(s) -
Papakonstantinou E.,
Prasse A.,
Schacht V.,
Kapp A.,
Raap U.
Publication year - 2016
Publication title -
journal of the european academy of dermatology and venereology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.655
H-Index - 107
eISSN - 1468-3083
pISSN - 0926-9959
DOI - 10.1111/jdv.13657
Subject(s) - pirfenidone , medicine , tolerability , rash , dermatology , itching , adverse effect , erythema , idiopathic pulmonary fibrosis , phototoxicity , gastroenterology , surgery , lung , biochemistry , chemistry , in vitro
Background Idiopathic pulmonary fibrosis ( IPF ) is a progressive and lethal pulmonary disease with an estimated 5‐year survival of approximately 20%. Pirfenidone is a novel orally available antifibrotic agent that reduces disease progression and improves survival of patients with IPF . The most common adverse effects of pirfenidone include gastrointestinal symptoms, hepatic dysfunction or skin photosensitivity and rash. A 64‐year‐old male patient presented in our clinic with a strong generalized exfoliative erythema and intense itching accompanied by fatigue and mild fever after a mild sun exposure for 5 days during holidays in Turkey. The patient had been diagnosed with IPF 2 months ago and 1 month later he started a therapy with pirfenidone with good tolerability. Objective In this report, we noted a severe phototoxic reaction under treatment with pirfenidone which underlies the potential phototoxic effect of this drug besides the already reported photosensitivity. Methods Routine laboratory tests and a skin biopsy were performed. Results Laboratory tests indicated increased markers of inflammation. The skin biopsy showed a perivascular lymphocytic inflammatory infiltrate, ballooning of keratinocytes with increased apoptosis. These findings were most consistent with a severe phototoxic reaction to pirfenidone which had been directly discontinued. The patient was started on oral methylprednisolone 100 mg/day which was gradually tapered off along with topical corticosteroids (mometasone furoate 0.1% cream) and oral antihistamines. This treatment led to a slow but complete resolution of the skin lesions within 20 days. Conclusion To our knowledge, this is the first reported case of a severe phototoxic reaction during treatment with pirfenidone. Our aim by presenting this case is to increase the awareness of clinicians for severe phototoxic effects of oral pirfenidone.