HISTORY

Mr A, aged 52, who had undergone bilateral total hip replacement 4 years ago for limb inequality, presented with painful ulcers of the lower limbs, pubis and back that had been developing for 10 months with weight loss. The patient reported a major worsening after a surgical debridement.

Physical examination found a patient in good general health with multiple large well-limited ulcers, deep-lying and purulent, surrounded by a purplish border with an underlying purulent rim. The ulcers were located on the dorsum of both feet, mutilating the toes, on both legs, across the two scars from the hip replacement, on the lower back and on the pubis, extending to the base of the penis with major psychological impairment (Fig. 1). The examination revealed no subcutaneous nodules, and the vascular assessment was normal.

Figure 1: Deep-lying ulcers with purplish undermined borders on the surgical scars, pubis, lower limbs and the back.

Laboratory work-up showed elevated erythrocyte sediment rate, C-reactive protein. Anti-neutrophil-cytoplasmic-antibody were negative. Further investigations allowed to rule out any underlying hemopathy, chronic inflammatory bowel disease, rheumatologic or neoplastic conditions. Bacteriological and mycological cultures and stains on skin tissue were negative.

Histology revealed a polymorphic inflammatory infiltrate associated with epithelioid granulomas and eosinophilic necrosis, and the subsequent pathology reviews were in favor of a polymorphic infiltrate rich in neutrophils with the focal presence of macrophagic granulomas with no signs of vasculitis (Fig. 2).

Figure 2: Histopathological image showing an epidermal necrosis, a dense neutrophilic infiltrate and macrophagic granulomas (Hematein-Eosin stain, 200 magnification).

WHAT’S THE DIAGNOSIS

DIAGNOSIS AND DISCUSSION

After ruling out differential diagnosis, the anatomo-clinical correlation and the various investigations ultimately led to the diagnosis of pyoderma gangrenosum. Pyoderma gangrenosum (PG) is a neutrophilic dermatosis resulting from a complex mechanism.

Maverakis et al. 2018 criterias of Delphi consensus are still recommended for positive diagnosis [1]. The major criterion implies the presence of a neutrophilic infiltrate of the ulcerative borders. Additional minor criteria intend to exclude evidence of infection, pathergy phenomenon, history of bowel inflammatory disease or inflammatory arthritis, history of papule, pustule or vesicle that ulcerates within 4 days of appearance, peripheral erythema and undermined border, multiple ulcerations and at least one on lower extremity, a cribriform wound healing and responsiveness to immunosuppressive therapy [2]. Our patient met the requisite criteria for the diagnosis to be retained, except that no disease association could be identified.

Another peculiarity of our patient was the puzzling histology due to the presence of granulomas, thus raising a differential diagnosis challenge with infectious causes and granulomatous polyangiitis.

Moreover, granulomatous subtype of PG is known to be relatively indolent, with a favorable response to local treatments [3]. This contrasts with the mutilating ulcers observed in our patient, which required systemic therapies.

Given the extensive nature of our patient’s condition, immunosuppressive treatment with corticosteroids dapsone and colchicine was initiated, yet the response remained unsatisfactory with relapses and side effects of long-term steroid use.

Finally, the administration of 160 mg adalimumab at week 0, 80 mg at week 2, and then 40 mg each week according to the protocol of Yamasaki et al. [4] resulted in a complete healing with a marked improvement in quality of life (Fig. 3).

Figure 2: Complete healing of the ulcers previously described after treatment with adalimumab.

PG remains a challenging condition to diagnose and manage effectively. This case was exceptional from a diagnostic and therapeutic point of view, hence the importance of clinico-pathological correlation and the use of combined therapies to achieve optimal outcomes.

Consent

The examination of the patient was conducted according to the principles of the Declaration of Helsinki.

Copyright by authors of this article. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

Request permissions
If you wish to reuse any or all of this article please use the e-mail (contact@odermatol.com) to contact with publisher.

Related Content:

Related Articles	Search Authors in
	Saadia Boughaleb http://orcid.org/0009-0002-2656-443X Zakia Douhi http://orcid.org/0009‑0004‑0702‑4037 Imane Fadlallah Meryem Soughi http://orcid.org/0009‑0002‑9985‑5131 Sara Elloudi http://orcid.org/0000-0002-5942-441X Layla Tahiri El Ousrouti Hanane Baybay http://orcid.org/0000-0003-3455-3810 Layla Chbani Fatima Zahra Mernissi http://orcid.org/0000‑0002‑5360‑337X

Rights and permissions

This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.