Multifocal and refractory pyoderma gangrenosum: Possible role of cocaine abuse

Abstract

Pyoderma gangrenosum is a rare and severe inflammatory skin condition. There are different variants, including generalised and atypical forms, but the most common presentation is an enlarging ulcer on the lower extremities. Treatment can represent a challenge for physicians and there are no guidelines based on randomised controlled trials. We report an exceptional case of widespread and refractory pyoderma gangrenosum in a middle-aged woman where cocaine use may have played a role. Treatment with i.v. pulse corticosteroids and cyclosporine was ineffective, and disease control was obtained with oral corticosteroids together with mycophenolic acid, infliximab and abstinence from cocaine consumption. There was a temporal relationship between disease outbreaks and cocaine consumption and improvement after its discontinuation. In the present case such clinical severity without associated pathology and the temporal association with cocaine abuse raises the possibility of cocaine playing an aetiological role as well as accounting for therapy resistance.

Cocaine‐induced pyoderma gangrenosum‐like lesions

“…While a predominantly dermal diffuse neutrophilic infi ltrate is the most relevant fi nding in CIPG, fi brinoid necrosis or even LCV may be present as well (Table 1 ), similar to what is observed in RP. Some authors claim that these histological similarities reinforce the pathogenic role of levamisole in both disorders [ 1,10,11 ] . Nevertheless, as commented above, levamisole has not been studied in all CIPG cases [ 1,8,9 ] .…”

“…Histopathologically, in addition to the diffuse dermal infi ltrate of neutrophils typical of classic forms of PG, distinctive traits such as fi brinoid necrosis, LCV and thrombotic vasculopathy have been described (Table 1 ). Furthermore, the reported immunological profi le resembles that of cases of RP induced by levamisole (Table 1 ) [ 1,[7][8][9][10][11][12][13][14] . Additionally, many patients with CIPG lesions presented with concomitant RP [ 1 ] .…”

“…Reinforcing this statement, patients 2 and 3 had C3 deposits in the DIF, but no levamisole was detected in the hair and no LCV was found in the histological examination, making the immunological mechanism independent of the presence of levamisole or a specifi c histological pattern. Perhaps, as Baliu-Piqué et al recently reported, a certain "autoimmune predisposition" may contribute to drug abusers developing CIPG after consuming cocaine, whether it is adulterated or not [ 11 ] .…”

“…Additionally, many patients with CIPG lesions presented with concomitant RP [ 1 ] . These comorbidities, together with the high incidence of levamisole-adulterated cocaine, led authors to argue that most cases of CIPG might also be associated with levamisole [ 1,10,11 ] . However, determination of this adulterant was performed in only one patient with CIPG [ 13 ] (Table 1 ).…”

Multiple Pyoderma Gangrenosum Ulcers Associated with Cocaine Abuse

“…3 Endothelial damage due to the direct toxic effect of cocaine and vasculopathy caused by levamisole have been postulated to be the major pathophysiological mechanisms in these types of cases. 4 The therapeutical approach may be complicated; the severity of PG influences the choice of the initial therapy. Systemic therapy-such as corticosteroids, immunosuppressive therapy or even biological therapy-is needed to achieve remission in cases of extensive PG; cessation of cocaine use is also imperative.…”