Hidradenitis Suppurativa and Concomitant Pyoderma Gangrenosum Treated With Infliximab Patricia F. Groleau, MD; Anna L. Grossberg, MD; Anthony A. Gaspari, MD Practice Points Pyoderma gangrenosum (PG) and hidradenitis suppurativa (HS) are rare chronic inflammatory dermato- ses that may coexist in the same patient. Infliximab may represent an effective therapeutic option for the treatment of concurrent PG and HS that is refractory to conventional therapies. copy Pyoderma gangrenosum (PG) and hidradenitis notyoderma gangrenosum (PG) and hidradenitis suppurativa (HS) are rare chronic inflammatory suppurativa (HS) are rare chronic inflamma- dermatoses of unknown etiologies that often Ptory dermatoses of unknown etiologies that are refractory to conventional treatments. DoThe often are refractory to conventional treatments. therapeutic benefits of tumor necrosis factor α Multiple case reports have described the coexistence (TNF- α) inhibitors have been reported in patients of these 2 diseases in the same patient.1-11 The diag- with refractory PG or HS. The copresentation of nosis of PG commonly followed the diagnosis of HS these 2 diseases has previously been described by 5 months to 30 years in several of these cases, in several cases in the literature and may present suggesting that HS may have triggered a possible a therapeutic challenge. We present the case of underlying inflammatory process that resulted in a 51-year-old man who developed widespread subsequent development of PG. When presenting inflammatory ulcers affectingCUTIS approximately 50% in the setting of preexisting HS, PG lesions have of the body surface area and subsequent chronic occurred both in the same sites of the body affected debilitation from severe pain. He was ultimately by HS as well as in distinct sites such as the legs.1 diagnosed with concurrent PG and HS. Both Although the treatment of either PG or HS alone diseases remitted in response to treatment with can be difficult, the combination of these 2 diseases infliximab, which resulted in complete restora- presents a further therapeutic challenge. Tumor necro- tion of skin integrity and resolution of his chronic sis factor α (TNF-α) has been implicated in the patho- severe pain. genesis of both of these diseases, and several cases have Cutis. 2015;95:337-342. demonstrated great potential for the use of TNF-α inhibitors, particularly infliximab, in the treatment of resistant cases of each of these conditions.2,12 We report a case of severe concurrent PG and HS in a 51-year-old man that was refractory to other treatments but clini- cally remitted in response to infliximab therapy. From the Department of Dermatology, University of Maryland School of Medicine, Baltimore. The authors report no conflict of interest. Case Report Correspondence: Anna L. Grossberg, MD, 419 W Redwood St, A 51-year-old man was transferred to our institution Ste 240, Baltimore, MD 21201 ([email protected]). from an outside hospital where he had presented WWW.CUTIS.COM VOLUME 95, JUNE 2015 337 Copyright Cutis 2015. No part of this publication may be reproduced, stored, or transmitted without the prior written permission of the Publisher. HS and Concomitant PG with fevers and a worsening rash that had prevented mentioned areas (Figures 1A and 1B), and deeper him from ambulating for several days secondary to purulent sinus tracts were noted in the axillae severe pain. At the outside facility, he was noted to and extending onto the chest (Figure 1C). A have extensive ulcerations with granulation tissue biopsy from the chest showed folliculitis with a on the scalp, face, sacrum, buttocks, and bilateral prominent plasmacytic infiltrate consistent with legs. A skin biopsy performed at the outside facil- HS. Cultures from the sinus tracts showed abun- ity revealed a neutrophilic dermal infiltrate with dant growth of Enterobacter aerogenes, Klebsiella abscesses and granulation tissue consistent with PG. pneumoniae, Enterobacter cloacae, and Proteus He was transferred to our institution for continued mirabilis. The patient was subsequently treated local wound care, corticosteroid administration, and with intravenous ampicillin-sulbactam and van- hyperbaric oxygen therapy. comycin as well as oral prednisone 80 mg daily On presentation at our institution, continued for 2 weeks with only mild improvement of the ulcerative skin lesions were noted in the previously lesions. The prednisone was gradually tapered to copy not DoA B CUTIS C Figure 1. On initial presentation, extensive areas of ulceration with variable amounts of granulation tissue and rusting were noted on the scalp (A) and legs (B). Sinus tract formation also was evident in the neck (A) and axilla (C). 338 CUTIS® WWW.CUTIS.COM Copyright Cutis 2015. No part of this publication may be reproduced, stored, or transmitted without the prior written permission of the Publisher. HS and Concomitant PG a dose of 10 mg daily in preparation for a trial of erosions on the head, neck, and chest. The patient infliximab. During the early course of his ther- reported no adverse effects during or after each apy, subsequent cultures from draining sinus tracts treatment. Following completion of the 3 inflix- grew Pseudomonas aeruginosa and Acinetobacter imab infusions, the skin lesions showed considerable baumannii, requiring concomitant treatment with improvement, with only 1 draining lesion remaining oral antimicrobials including doxycycline, on the left temple and only erythematous plaques sulfamethoxazole-trimethoprim, ciprofloxacin, remaining on the scalp, upper back, and axillae. and amoxicillin–clavulanic acid for control of Maintenance infusions were continued every 8 weeks the superinfection. with an increased infliximab dose of 7.5 mg/kg. The patient received 3 induction infusions of After 1 year of treatment, the lesions had healed infliximab at a dosage of 5 mg/kg per treatment at to form cicatrices with no evidence of erythema, weeks 0, 2, and 6. At the time of the first treatment, drainage, or infection (Figure 2). Doxycycline and he was noted to have numerous crusted draining prednisone were discontinued, and the infliximab copy not Do A B CUTIS C Figure 2. After 11 infusions with infliximab (dose range, 5–7.5 mg/kg), all previously affected areas showed resolu- tion with cicatrices and postinflammatory hyperpigmentation (A–C), with no evidence of ulceration or active sinus tract formation. WWW.CUTIS.COM VOLUME 95, JUNE 2015 339 Copyright Cutis 2015. No part of this publication may be reproduced, stored, or transmitted without the prior written permission of the Publisher. HS and Concomitant PG dose was decreased to 5 mg/kg per infusion every Hidradenitis suppurativa is characterized by recur- 8 weeks. Following sustained improvement after rent skin abscesses, sinus tract and fistula for- 2 infusions at this lower dose, infliximab was suc- mation, and subsequent fibrosis with bacterial cessfully tapered to 2.5 mg/kg every 8 weeks for overgrowth as a common secondary process.14 The 2 doses and then was subsequently discontinued; pathogenesis of HS remains poorly understood, but however, the patient’s disease relapsed approxi- histology typically shows a nonspecific inflamma- mately 7 months after discontinuation of the tory process with or without concomitant infec- infliximab and was immediately resumed at a dose tion.15 Treatment of HS is complex and usually is of 5 mg/kg per infusion every 8 weeks. He has transiently effective at best.14 In 2012, Rambhatla remained disease free on this dose to date. et al16 discussed the efficacy of various treatments of HS, including systemic and topical antimicro- Comment bial agents (eg, clindamycin-rifampicin combina- Pyoderma gangrenosum is a chronic inflammatory tion treatment, tetracycline, topical clindamycin ulcerative skin condition that most commonly phosphate, isotretinoin, dapsone), antiandrogenic occurs on the lower legs.3 In its most common agents, biologic agents (eg, infliximab, etanercept, form, PG lesions typically begin as tender ery- adalimumab, efalizumab), laser surgery (CO2 laser, thematous nodules or pustules that evolve into Nd:YAG laser), and excisional surgery of sinus enlarging painful ulcers with raised, undermined, tracts. Other therapies include cryotherapy, photo- violaceous borders.13 Biopsy specimens taken from dynamic therapy, finasteride, zinc gluconate, topi- the edges of the lesions typically show a diffuse cal resorcinol, and acitretin.16 neutrophilic infiltrate, and pseudoepitheliomatous Both PG and HS are categorized as chronic hyperplasia also may be seen. Lesions tend to per- inflammatory disorders with nonspecific histopatho- sist for months to years, ultimately healing as crib- logic findings.copy Although the etiologies of these riform scars. The etiology of PG is unknown and 2 disorders remain poorly understood, several case its pathogenesis is poorly understood.13 Pyoderma reports have suggested an association between PG gangrenosum is associated with an underlying sys- and HS.1,4 In many of the reported cases of con- temic disease in approximately 50% of cases, most currentnot HS and PG, HS preceded the diagnosis of commonly inflammatory bowel disease, hemato- PG by several years and no correlation in disease logic malignancies, and inflammatory arthritis.3 An activity was observed between the 2 conditions.1-4,6-8 underlying immunologic abnormality is therefore Additionally, the clinical triad of PG, acne, and postulated to contribute to the pathogenesisDo of suppurative hidradenitis,