A case of recalcitrant acute generalized exanthematous pustulosis with Sjogren's syndrome: Successfully treated with low‐dose cyclosporine

Abstract

Acute generalized exanthematous pustulosis (AGEP) is a reaction attributed mainly to drugs. Hydroxychloroquine is a rare cause of AGEP especially used in rheumatology and dermatology. Systemic corticosteroids are the first‐line treatment agents in AGEP. But cyclosporine can be a good choice for patients resistant to systemic corticosteroid treatment in AGEP.

Hydroxychloroquine‐induced acute generalized exanthematous pustulosis: a series of seven patients and review of the literature

“…However, longer durations for resolution have been reported in HCQ‐induced AGEP ranging from 7 to 81 days 16,17 . Persistence of symptoms beyond the usual course could be explained by the lengthy half‐life of HCQ, approximately 40–50 days 18 . In prolonged cases, topical steroids may be useful for treatment of pruritus and inflammation 19 .…”

Generalized pustular figurate erythema: A newly delineated severe cutaneous drug reaction linked with hydroxychloroquine

“…There is another rare acute severe generalized disorder, one usually characterized as AGEP, but with an onset of 2 to 3 weeks (range 4‐27 days) rather 1 day after initial drug exposure, typically due to hydroxychloroquine, more severe, more difficult to treat, with a longer duration, and recognized as likely having a different pathogenic mechanism from the usual type of AGEP 10 . This perplexing disorder has been described as atypical AGEP, 11,12 recalcitrant AGEP, 13,14 pustular DRESS syndrome, 15 AGEP/SJS overlap, 16 AGEP/TEN overlap, 17,18 and Sweet's syndrome following hydroxychloroquine 19‐21 . We delineate and highlight it as generalized pustular figurate erythema (GPFE).…”

“…Clinical examination reveals an abrupt onset of a pruritic eruption representing a severe cutaneous drug reaction with fever and neutrophilic leukocytosis 11‐21 . GPFE may be first evident as erythematous papules and plaques on the face with facial edema and widespread urticarial or edematous plaques scattered over the entire body, with development of nonfollicular pustules atop what evolve into erythematous and sometimes atypical targetoid erythema multiforme‐like plaques converging into annular and arcuate patterns prominent on the trunk and extremities (Figure 1).…”

“…Treatments have been varied 11‐21,24‐26 . The first line therapy is topical and systemic steroids, which may be followed by cyclosporine if GPFE is not responsive 13,14,21 . Other options include potent topical steroids with oral dapsone or etretinate 25 .…”

Acute generalized exanthematous pustulosis induced by hydroxychloroquine prescribed for COVID-19

“…HCQ has numerous skin side effects including maculopapular rash, cutaneous hyperpigmentation, pruritus, AGEP, Stevens-Johnson syndrome or toxic epidermal necrolysis, hair loss, and stomatitis, as previously reported. [3][4][5] In AGEP, the average duration of drug exposure before onset of the symptoms depends on the causative drug. Antibiotics such as amoxicillin consistently have a short latency of 24 to 72 hours, whereas other medications, including HCQ, are often associated with latencies around 10 to 12 days or longer (16.2 days for HCQ in our review).…”