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Pyoderma Gangrenosum Pyoderma Gangrenosum CAZURI CLINICE CLINICAL CASES PYODERMA GANGRENOSUM PYODERMA GANGRENOSUM GABRIELA RALUCA MIULESCU*, MARIA MAGDALENA CONSTANTIN**,***, **MIHAI CRISTIAN DUMITRAªCU***,****, RÃZVAN-COSMIN PETCA***,*****, ANA MARIA ALEXANDRA STÃNESCU***, FLORICA ªANDRU*,*** Rezumat Summary Prezentãm cazul unui pacient în vârstã de 65 de ani, We present the case of a 65 years old man, with cu multiple comorbiditãþi, care a dezvoltat o ulceraþie multiple comorbidities, who reffered to our Dermatology ovalarã, cu margini eritemato-violacee, cu baza necroticã, Clinic for the evaluation, of an oval ulceration, with localizatã la nivelul gambei stângi. Întrucât aceasta nu a erythematous-violet margins, with necrotic base, located at rãspuns la tratamentul cu dermatocorticoizi, ºi luând în the level of the left calf. Because the treatment with considerare aspectul clinic, vârsta ºi antecedentele dermatocorticoids was ineffective, and considering the personale patologice, s-a ridicat suspiciunea clinicã de clinical appearance, age and personal history, the clinical pyoderma gangrenosum. S-a efectuat biopsie cutanatã cu suspicion of pyoderma gangrenosum has been raised. Skin examen histopatologic, care a relevat: infiltrate neutrofilice biopsy was performed with histopathological examination, cu aspect supurativ, fibrozã, þesut de granulaþie; coloraþie which revealed: suppurative neutrophilic infiltrate, fibrosis, PAS ce nu a evidenþiat elemente micotice, susþinându-se granulation tissue; PAS staining that did not show a fungal astfel diagnosticul de pyoderma gangrenosum. Tratamentul element, thus supporting the diagnosis of pyoderma sistemic a constat în dapsonã ºi corticoterapie, cu evoluþie gangrenosum. Systemic treatment with dapsone and favorabilã. corticosteroids was initiated, with favorable evolution. Pyoderma gangrenosum (PG) este o patologie Pyoderma gangrenosum (PG) is a rare, chronic, cutanatã ulcerativã, rarã, cronicã, caracterizatã prin ulcerative skin disease, characterized by rapid progression progresia rapidã a unui ulcer dureros, cu margini to a painful ulcer, with irregular, purple margins. The neregulate, violacee. Diagnosticul se stabileºte atât prin diagnosis is established by histopathological examination, examen histopatologic, cât ºi prin excluderea altor cauze de perhaps by excluding other causes of skin ulceration. Also, ulceraþie cutanatã. De asemenea, fenomenul de patergie the phenomenon of patergia precedes the appearance of the precede deseori apariþia leziunii. injury. Managementul unor astfel de pacienþi este unul Managing such patients is extremely difficult, because extrem de dificil, deoarece recãderile sunt frecvente, iar they are common and care pathologies are associated with patologiile care se asociazã numeroase. the number. Cuvinte cheie: pyoderma gangrenosum, boalã Crohn, Keywords: pyoderma gangrenosum, Crohn’s disease, ulceraþie, dapsona, corticosteroizi. ulceration, dapsone, corticosteroids. Intrat în redacþie: 04.11.2019 Received: 04.11.2019 Acceptat: 07.12.2019 Accepted: 07.12.2019 * Clinica Dermatologie, Spitalul Clinic Universitar de Urgenþã ,,Elias”, Bucureºti, România Department of Dermatology, ”Elias” University Emergency Hospital, Bucharest, Romania ** Clinica II Dermatologie, Spitalul Clinic Colentina, Bucureºti, România Department of Dermatology II, Colentina Clinical Hospital, Bucharest, Romania *** Universitatea de Medicinã ºi Farmacie ,,Carol Davila”, Bucureºti, România ”Carol Davila” University of Medicine and Pharmacy, Bucharest, Romania **** Clinica de Obstetricã ºi Ginecologie, Spitalul Universitar de Urgenþã, Bucureºti, România Department of Obstetrics and Gynecology, University Emergency Hospital, Bucharest, Romania ***** Clinica de Urologie, Spitalul Clinic ,,Prof. DR. Theodor Burghele”, Bucureºti, România Department of Urology, ”Prof. Dr. Theodor Burghele” Clinical Hospital, Bucharest 253 DermatoVenerol. (Buc.), 64(4): 253-259 Introducere Introduction PG reprezintã o dermatozã neutrofilicã rarã, PG is a rare, non-infectious, recurrent non-infecþioasã, recurentã, cu aspect morfologic neutrophilic dermatosis with distinct distinct, ºi asociatã, de cele mai multe ori, cu o morphological appearance, and most often patologie sistemicã [1]. Existã patru forme clinice associated with systemic pathology [1]. There are de PG: ulcerativã, buloasã, pustuloasã, granulo- four clinical forms of PG: ulcerative, bullous, matoasã superficialã. Cel mai frecvent, aceastã pustular, superficial granulomatous. Most dermatozã debuteazã printr-o pustulã, localizatã commonly, this dermatosis begins with a pustule, pe un fond eritemato-violaceu, printr-un nodul located on an erythematous-purple background, eritematos, sau printr-o bulã. În timp, aceastã through an erythematous node, or through a leziune evolueazã într-o ulceraþie cu margine bubble. Over time, this lesion develops into an necroticã, ºi cu baza purulentã sau vegetativã [2]. ulceration with a necrotic margin, and with a Conform unor studii, incidenþa a fost purulent or vegetative base [2]. raportatã la 3-10 cazuri la 1 milion de persoane, According to some studies, the incidence was pe an. PG se poate manifesta la orice vârstã, însã reported in 3-10 cases per 1 million people, per cel mai frecvent apare la indivizi între 40 ºi 60 de year. PG can occur at any age, but most ani. De asemenea, aceastã patologie dermato- commonly occurs in individuals between 40 and logicã este mai des întâlnitã la femei [3-7]. 60 years. Also, this dermatological pathology is Etiopatogeneza PG nu este pe deplin more common in women [3-7]. elucidatã. În prezent, existã mai multe teorii prin The etiopathogenesis of PG is not fully care aceastã dermatoza s-ar produce. În primul elucidated. Currently, there are several theories rând, fiind o dermatozã neutrofilicã, este by which this dermatosis would occur. First, recunoscut rolul disfuncþiei neutrofilelor în being a neutrophilic dermatosis, the role of declanºarea bolii. Acest fapt este susþinut ºi de neutrophil dysfunction in triggering the disease rãspunsul benefic al PG la agenþi anti-neutrofilici, is recognized. This fact is also supported by the cum ar fi dapsona [8]. În al doilea rând, factorul beneficial response of PG to anti-neutrophilic genetic joacã un rol extrem de important: au fost agents, such as dapsone [8]. Second, the genetic raportate cazuri familiale de PG [9, 10]. Conform factor plays an extremely important role: familial literaturii de specialitate, sindromul PAPA se cases of PG have been reported [9, 10]. According manifestã prin: artritã piogenicã sterilã, PG ºi to the literature, PAPA syndrome is manifested acnee. Gena implicatã în dezvoltarea acestui by: pyogenic arthritis, PG and acne. The gene sindrom este localizatã pe cromozomul 15q, involved in the development of this syndrome is gena PSTPIP1/CD2BP1 care codeazã prolina/ located on chromosome 15q, the PSTPIP1 / serina/proteina 1 care interacþioneazã cu fosfatul CD2BP1 gene that encodes proline / serine / treoninei. Se considerã cã tocmai mutaþiile din threonine phosphatase-interacting protein. It is aceastã genã sunt cele care declanºeazã ºi considered that it is precisely the mutations in menþin procesul inflamator [11, 12]. Dupã cum this gene that trigger and maintain the am menþionat anterior, procesul inflamator, ºi inflammatory process [11, 12]. As mentioned anume asocierea cu boli inflamatorii sistemice above, the inflammatory process, namely the (boalã inflamatorie intestinalã, artritã) este association with systemic inflammatory diseases frecventã în cazul pacienþilor cu PG. Studiile (inflammatory bowel disease, arthritis) is demonstreazã creºterea producþiei de citokine common in patients with PG. Studies show (IL-8, IL-23). Ca ºi în cazul dapsonei, rãspunsul increased cytokine production (IL-8, IL-23). As terapeutic favorabil la terapii biologice (agenþi with dapsone, the favorable therapeutic response anti-TNF-α), susþine rolul TNF-α în etiologia bolii to biological therapies (anti-TNF-α agents) [8, 13, 14]. supports the role of TNF-α in the etiology of the Diagnosticul PG este în primul rând unul de disease [8, 13, 14]. excludere, realizându-se diagnosticul diferenþial The diagnosis of PG is primarily one of cu alte ulceraþii, întrucât aspectul clinic, exclusion, the differential diagnosis being made examinãrile paraclinice ºi analizele de laborator with other ulcerations, since the clinical aspect, 254 DermatoVenerol. (Buc.), 64(4): 253-259 nu sunt specifice acestei dermatoze. Tocmai din the paraclinical examinations and the laboratory aceastã cauzã, se recomandã efectuarea unei analyzes are not specific to this dermatosis. anamneze atente (fenomene de patergie în Precisely for this reason, it is recommended to istoricul pacientului), examen obiectiv general perform a careful history (patergia phenomena in (asocierea unor posibile patologii), biopsie the patient’s history), general objective exa- cutanatã ºi examen histopatologic [8]. mination (association of possible pathologies), skin biopsy and histopathological examination Prezentare de caz [8]. Prezentãm cazul unui pacient în vârstã de 65 Case presentation de ani, cunoscut cu multiple comorbiditãþi, care s-a prezentat în clinicã noastrã pentru o ulceraþie We present the case of a patient aged 65 years, ovalarã, dureroasã, localizatã la nivelul gambei known with multiple comorbidities, who stângi, în evoluþie de 4 luni. Tratamentul anterior presented in our clinic with an oval ulceration, prezentãrii în clinica noastrã, cu dermato- painful, located at the level of the left
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