Pyoderma gangrenosum – a real management challenge: clinical case

Abstract

Pyoderma gangrenosum (PG) is a rare neutrophillic skin disorder, with a chronic evolution with chronic evolution. PG is estimated to affect up to 10 people per 1 million population. [1] Clinically, it manifests with pustular, painful nodular lesions, that progressively enlarge and break down to form an ulcer with violet and intensely erythematous edges. PG can be associated with inflammatory bowel disease, arthritis, and malignant tumors, sometimes precedes these disorders or may evolve without an underlying disease. The following clinical forms of PG are distinguished: ulcerative, bullous, pustular, vegetative, and peristomal. The diagnosis is established based on clinical data and histopathological examination, the main value of which is the exclusion of other causes of ulceration. Systemic treatment targets immunosuppressive agents (corticosteroids, ciclosporin); biological agents, and sulfones. Topical treatment is aimed to stop the expansion of the inflammatory process, favoring the healing of ulcers, and preventing superinfection. The objective of the study is to emphasize the importance of establishing the diagnosis of PG as early as possible to avoid management errors. Case presentation A 58-year-old female patient presented for a consultation in the Dermatology department for the appearance of skin lesions, located on the right forearm, left lower limb, and pathergic lesion in the gluteal region. The patient reported that the condition started 3 years ago, with the appearance of nodular lesions, later - ulcerative on the lower legs and submammary. Antibacterial treatment and performing surgical drainage, have been ineffective. Pathological antecedents were not documented. The clinical examination revealed: erythematoedematous lesions with dimensions between 4-11 cm in diameter, purulent exudate, with an ulcerated surface with a necrotic periphery, as well as cribriform atrophic scars with an irregular outline. Lesions, accompanied by moderate-severe, permanent pain. Paraclinical: accelerated ESR and biological inflammatory syndrome - PCR 4+. Bacteriological and mycological investigations were carried out from the contents of the wound – without bacterial or fungal growth. Histopathological examination: massive neutrophilic infiltration in the dermis and modest lymphocytic inflammatory infiltrate. Medium-dose systemic corticosteroids with slow tapering and sulfones were used. Topical treatment included: corticosteroids and hydrocolloid dressings. Discussions Following paraclinical examinations, associated systemic diseases and precipitating factors were not identified, which was revealed in only 30% of PG cases. PG lesions tend to occur in areas of minor trauma, given the phenomenon of pathergy. Differential diagnosis is the key to success. Initially, PG takes the appearance of painful nodules, and pustules, this being confused with furunculosis, abscesses, and in the late stages – idiopathic ulcers with the following incorrect therapeutic strategy. The diagnosis of PG was established based on 2 major criteria and 3 minor criteria from those established. [2] The indicated, progressive treatment led to the reduction of the ulcers in size, with the formation of cribriform atrophic scars and the absence of new lesions after 1 year of follow-up. Conclusions PG has mimicked various dermatoses over a long period. The diagnosis was difficult, being one of exclusion. Its correct establishment was decisive in choosing the therapeutic strategy and tempering the skin’s pathological process.