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Article in English | WPRIM | ID: wpr-984445


Introduction@#Pyoderma gangrenosum (PG) is a rare inflammatory disease with unknown etiology. Ulcerative PG presents with a rapidly enlarging painful ulcer with erythematous and undermined border often misdiagnosed as infection, vascular disorder, malignancy, and other inflammatory disease. Hence, this poses a diagnostic challenge for clinicians leading to a delay in the management and significant morbidity. The treatment of PG is equally challenging due to the rarity of the disease and the scarcity of clinical trials. Currently, there are no clinical practice guidelines for the management of PG. @*Case Report@#Our patient presented with multiple large ulcers with erythematous and undermined borders over the chest, abdomen, and the lower back. Cribriform scars and contractures were noted as well. She underwent several sessions of surgical debridement and was given different broad-spectrum antibiotics with noted worsening of the lesions. Due to extensive involvement of the disease, her quality of life has been significantly affected. A diagnosis of PG was made after the biopsy showed predominantly neutrophilic infiltrate. Prednisone 1mg/kg/day and clobetasol propionate ointment were initiated with significant decrease in pain and size of the ulcers after one month of therapy. Doxycycline was used as an adjunct therapy with excellent response.@*Conclusion@#Pyoderma gangrenosum is a rare, debilitating disease that remains a diagnostic dilemma. The worsening of ulcers despite surgical debridement and antibiotics is a clue that should prompt clinicians to consider PG. This case highlights the important role of dermatology in individuals who present with non-healing chronic ulcers because as seen in this case, not all ulcers are just ulcers.

Pyoderma Gangrenosum , Ulcer
Actual. SIDA. infectol ; 30(110): 33-36, 20220000. fig
Article in Spanish | LILACS, BINACIS | ID: biblio-1413650


La histoplasmosis diseminada es una infección oportunista frecuente en los pacientes VIH/sida. Frecuentemente se encuentra compromiso cutáneo, siendo las formas clínicas más comunes el aspecto moluscoide, variceliforme y papulonecrótico.Se presenta el caso de una paciente con una forma cutánea atípica de histoplasmosis diseminada similar a pioderma gangrenoso cuyo diagnóstico fue realizado mediante escarificación.Es importante mantener la sospecha clínica en estas presentaciones atípicas y reforzar la utilidad de la escarificación de lesiones cutáneas como método rápido y efectivo para su diagnóstico.

Disseminated histoplasmosis is a frequent opportunistic infection in HIV/AIDS patients. Cutaneous manifestations are common, the most frequent are molluscum contagio-sum-like lesions, varicelliform eruption and papulo-necro-tic lesions.We present a case of an atypical disseminated cutaneous histoplasmosis with pyoderma gangrenosum-like ulcers, diagnosed by direct examination of tissue smear.It is important to have a high clinical suspicion of this atypical presentation and to emphasize the usefulness of skin smear as a simple and effective test to establish its diagnosis

Humans , Female , Adult , Skin Ulcer , Opportunistic Infections/complications , HIV/immunology , Pyoderma Gangrenosum/diagnosis , Histoplasmosis/diagnosis
An. bras. dermatol ; 97(1): 121-123, Jan.-Feb. 2022. graf
Article in English | LILACS | ID: biblio-1360082
Article in English | WPRIM | ID: wpr-961153


Background@#Pyoderma gangrenosum is a rare ulcerative skin disease that can present as an ulcerative skin disease with the prominence of pain. The pathogenesis may be related to disruptions in the immune pathways. Targeted therapy is lacking and current treatment is largely empirical and consists of corticosteroids and cyclosporine first line. Early recognition can improve clinical outcomes.@*Case@#This case is a 67-year-old male diabetic who was admitted for a progressive ulcerative lesion on the right leg. Arterial Doppler studies and CT angiogram of the right lower extremity were normal. Blood and deep wound cultures of the lesion showed Klebsiella pneumoniae and Pseudomonas aeruginosa. Multiple antibiotic regimens were given with no improvement of the ulcerating lesions of the leg. Pain on the lesion remained persistent. The tissue biopsy of the lesion taken during debridement revealed that it was a pyoderma gangrenosum with dystrophic sclerosis. Oral prednisone at 1 mg/kg was added to the regimen which improved pain but the lesion did not improve. The persistence of the pain and progression to sepsis during the hospital course prompted the decision to do an above-knee amputation of the right leg. He was discharged improved.@*Conclusion@#Pyoderma gangrenosum is a rare non-infectious cause of an ulcerative lesion in the lower extremity. Diabetes is a strong risk factor for this disease. The course is prolonged with the possibility of secondary infections. Upon histopathologic confirmation, an anti-inflammatory regimen could help improve outcomes.

Pyoderma Gangrenosum , Diabetic Foot , Leg Ulcer , Inflammation , Anti-Bacterial Agents , Amputation, Surgical
Chinese Journal of Burns ; (6): 574-579, 2022.
Article in Chinese | WPRIM | ID: wpr-940963


Pyoderma gangrenosum (PG) is a rare chronic inflammatory non-infectious skin dermatosis, and there is no clear treatment guideline for this disease at home and abroad. There are a variety of clinical treatment methods for PG, including local therapy and systemic application of glucocorticoids, immunosuppressants, intravenous immuno- globulin, and biologics. Glucocorticoids are the first-line drugs commonly used in clinical practice, and immunosuppressants can be used alone or in combination with glucocorticoids. In recent years, more and more evidence has shown that biologics are a new trend in the treatment of PG, mainly including tumor necrosis factor α inhibitors, interleukin-1 (IL-1) inhibitors, IL-12/23 inhibitors, IL-17 inhibitors, rituximab, and small molecular inhibitors. This article summarizes the current status and latest progress in the treatment of PG, hoping to provide clinicians with ideas for the treatment of PG.

Humans , Biological Products , Glucocorticoids , Immunosuppressive Agents , Immunotherapy , Interleukin Inhibitors , Pyoderma Gangrenosum/drug therapy
Chinese Journal of Burns ; (6): 569-573, 2022.
Article in Chinese | WPRIM | ID: wpr-940962


Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis, closely associated with the immune system. Its pathogenesis is currently not clear. The lack of specificity in the clinical manifestations and histopathological changes of PG leads to a long clinical diagnosis cycle and even misdiagnosis, which is easy to delay treatment or promote the deterioration of ulcer wound. The diagnosis of this disease is still very difficult, which poses a great challenge to wound repair practitioners. This article reviews the research advances on the pathophysiology, clinical features, and diagnosis of PG in recent years, with the aim of providing reference for relevant clinical practitioners.

Humans , Pyoderma Gangrenosum/pathology
Chinese Journal of Burns ; (6): 506-511, 2022.
Article in Chinese | WPRIM | ID: wpr-940953


Objective: To explore the clinical features and treatment of pyoderma gangrenosum (PG). Methods: A retrospective observational study was conducted. From January 2012 to July 2021, 25 patients with PG who met the inclusion criteria were admitted to Beijing Fucheng Hospital, including 16 males and 9 females, with the age of onset of disease being 14 to 75 years. Among them, the classification of PG identified 17 cases of ulcerative type, 6 cases of pustular type, 1 case of proliferative type, and 1 case of bullous type. Six patients were accompanied with systemic diseases, while 19 patients were not accompanied with systemic diseases. At the same time of systemic treatment with glucocorticoids, dressing changes or surgical skin grafting was performed on the wounds. The results of laboratory and histopathological examinations, the overall curative effects and follow-up of patients, the wound healing time of patients with negative and positive microbial culture results of wound secretion specimens, and the curative effects of patients with and without systemic diseases were analyzed. Results: The results of blood routine examination of 19 patients were abnormal, and all the immunological indexes were normal in all the patients; the microbial culture results of wound secretion specimens were positive in 14 patients; and the histopathological examination results of ulcer boundary tissue in 15 patients with rapid wound progress were mainly local tissue inflammatory changes. The wounds were cured in 17 patients, mostly healed in 7 patients, and not healed in 1 patient. After one-year's follow-up, the PG in 3 patients relapsed due to self-discontinuation of medication after discharge, and the wounds were healed gradually after adjustment of medication, while the remaining patients had no relapse. The days of wound healing in 14 patients with positive microbial culture results of wound secretion specimens were 21-55 days, and the days of wound healing in 11 patients with negative microbial culture results in wound secretion specimens were 20-54 days. In the 6 patients with systemic diseases, the wounds of 3 patients were cured, and the wounds of the other 3 patients were mostly healed. In the 19 patients without systemic diseases, the wounds of 14 patients were cured, the wounds of 4 patients were mostly healed, and the wound of 1 patient was not healed. Conclusions: The laboratory examination and pathological manifestations of patients with PG lacks characteristics, and their clinical manifestations are rich and diverse, thus PG can be easily misdiagnosed. The glucocorticoids combined with immunosuppressive therapy have good effects on PG. Surgical intervention can be performed on the wounds. Specifically, excessive debridement is not recommended in the acute phase, but skin grafting can be performed in the contraction phase.

Adolescent , Adult , Aged , Female , Humans , Male , Middle Aged , Young Adult , Administration, Cutaneous , Glucocorticoids , Pyoderma Gangrenosum/therapy , Skin Transplantation , Wound Healing
An. bras. dermatol ; 96(5): 574-577, Sept.-Oct. 2021. graf
Article in English | LILACS | ID: biblio-1345148


Abstract Neutrophilic dermatoses encompass a wide spectrum of diseases characterized by a dense infiltration mainly composed of neutrophils. Neutrophilic dermatosis of the dorsal hands is currently considered a localized variant of Sweet syndrome. Cocaine abuse has been related to a wide range of mucocutaneous manifestations, including neutrophilic dermatoses such as pyoderma gangrenosum. The authors of this study present a patient with neutrophilic dermatosis of the dorsal hands, in which cocaine abuse was identified as a probable trigger.

Humans , Sweet Syndrome/diagnosis , Sweet Syndrome/chemically induced , Pyoderma Gangrenosum , Cocaine-Related Disorders/complications , Dermatitis , Neutrophils
An. bras. dermatol ; 96(2): 188-195, Mar.-Apr. 2021. tab, graf
Article in English | LILACS | ID: biblio-1248749


Abstract Pyoderma gangrenosum associated to the use of cocaine/levamisole is a rare condition associated to their consumption. Cocaine use is frequent in Colombia, and the substance is contaminated with levamisole, an anthelmintic that increases the psychotropic effects and enhances its side effects. We present three clinical cases of patients with ulcerated lesions, in which the diagnosis was pyoderma gangrenosum secondary to the use of cocaine contaminated with levamisole. This called the attention of the health staff to investigate the abuse of substances in gangrenous pyoderma and also evidence that the interruption of consumption was the basis of management.

Humans , Pyoderma Gangrenosum/chemically induced , Pyoderma Gangrenosum/drug therapy , Cocaine/adverse effects , Cocaine-Related Disorders/complications , Levamisole , Colombia
Rev. Hosp. Ital. B. Aires (2004) ; 41(1): 26-30, mar. 2021. ilus
Article in Spanish | LILACS | ID: biblio-1178336


El pioderma gangrenoso ampollar es una variedad infrecuente de pioderma gangrenoso, que se asocia en el 50-70% de los casos con trastornos oncohematológicos. Se comunica el caso de una paciente de 59 años, que consultó por fiebre y ampollas purpúricas de rápida progresión, con compromiso cutáneo mucoso. Con sospecha de una enfermedad neutrofílica, ampollar, o infección por gérmenes oportunistas, se realizó biopsia de piel para estudio histopatológico, inmunofluorescencia directa y cultivo. Los cultivos y la inmunofluorescencia directa fueron negativos, y la anatomía patológica reveló un denso infiltrado inflamatorio con predominio neutrofílico en dermis. Ante el diagnóstico de pioderma gangrenoso ampollar, se realizó una punción-aspiración de médula ósea cuyo resultado fue compatible con leucemia mieloide aguda. Se instauró tratamiento con corticosteroides sistémicos, a pesar de lo cual la paciente evolucionó desfavorablemente y falleció a los 15 días de su ingreso hospitalario. Este caso ilustra la asociación de esta enfermedad cutánea con trastornos oncohematológicos y el mal pronóstico que esto implica a corto plazo. (AU)

Bullous pyoderma gangrenosum is an infrequent type of pyoderma gangrenosum, associated with onco hematological diseases in 50-70% of cases. We present the case of a 59-year-old patient with fever and mucocutaneous hemorrhagic bullous of rapid progression. A biopsy for histopathology, direct immunofluorescence (DIF) and skin culture was made, considering the possibility of neutrophilic dermatoses, bullous dermatosis or an opportunistic infection. The results of both the culture and the DIF were negative. The histopathological examination of the specimen revealed a dense dermal polymorphic infiltrate composed primarily of neutrophils. Considering bullous pyoderma gangrenosum as a potential diagnosis, a bone-marrow biopsy was performed. This study revealed an acute myeloid leukemia. Although systemic corticosteroid therapy was begun, the patient presented an unfavorable evolution that led to her death 15 days after her admission at the hospital. This case shows the association between bullous pyoderma gangrenosum and onco hematological diseases. In addition, it highlights the poor prognosis related to these diseases in the short term. (AU)

Humans , Female , Middle Aged , Leukemia, Myeloid, Acute/pathology , Pyoderma Gangrenosum/diagnosis , Paraneoplastic Syndromes/pathology , Respiration, Artificial , Azacitidine/therapeutic use , Myelodysplastic Syndromes/pathology , Acyclovir/administration & dosage , Methylprednisolone/administration & dosage , Vancomycin/administration & dosage , Cardiotonic Agents/therapeutic use , Ceftazidime/administration & dosage , Amphotericin B/administration & dosage , Imipenem/administration & dosage , Sweet Syndrome/etiology , Pyoderma Gangrenosum/etiology , Pyoderma Gangrenosum/pathology , Pyoderma Gangrenosum/drug therapy , Adrenal Cortex Hormones/therapeutic use , Meropenem/administration & dosage
Article in English | WPRIM | ID: wpr-961869


Summary@#Pyoderma gangrenosum (PG) of the breast is a rare rapidly progressive neutrophilic dermatosis, which usually co-exists with severe underlying systemic conditions. A woman presented with a non-healing ulcer over her right breast with characteristic sparing of nipple-areola complex (Bork-Baykal phenomenon). It was diagnosed as pyoderma gangrenosum on the basis of clinico-pathological correlation and managed successfully with systemic corticosteroids and anti-inflammatory drugs along with wound care. The diagnosis and treatment of PG is challenging particularly at unusual sites given the paucity of robust clinical evidence and lack of consensus opinion regarding specific management guidelines. It is imperative that PG is considered as a clinical diagnosis in any patient with enlarging, sterile, necrotic lesions unresponsive to appropriate antibiotics. Early recognition of PG at rare locations can prevent devastating sequelae such as over-zealous surgical debridement and deep tissue infections associated with a chronic open wound leading to severe cosmetic morbidity.

Pyoderma Gangrenosum
J. Health Biol. Sci. (Online) ; 9(1): 1-4, 2021. ilus, tab
Article in Portuguese | LILACS | ID: biblio-1352364


Introdução: O pioderma gangrenoso é uma dermatose incomum, crônica e de apresentação clínica variável. Sua etiologia é indefinida, e seu tratamento não é bem estabelecido. O Adalimumabe é um anticorpo monoclonal humano contra o fator de necrose tumoral alfa (anti- TNF) que é raramente associado à disfunção cardíaca. Relato de Caso: Relatamos um caso de uma paciente com pioderma gangrenoso idiopático que, após duas doses de Adalimumabe, desenvolveu insuficiência cardíaca grave com miocardiopatia dilatada. Ela foi tratada com diurético de alça, betabloqueador e antagonista da enzima conversora da angiostensina. Após dois meses, encontrava-se sem queixas cardiorrespiratórias e com melhora da fração de ejeção. Conclusão: A miocardiopatia dilatada, apesar de ser manifestação rara do uso de fármacos anti -TNF deve ser considerada no diagnóstico diferencial de insuficiência respiratória após seu uso.

Introduction: Pyoderma gangrenosum is an unusual chronic dermatosis of mixed clinical presentation. Its etiology is unclear, and its treatment is not well established. Adalimumab is a human monoclonal antibody against the tumor necrosis factor alpha (anti TNF) which is rarely associated with cardiac dysfunction. Case Report: We report a case of a patient with idiopathic Pyoderma gangrenosum who developed severe heart failure with dilated myocardiopathy after two doses of Adalimumab. She was treated with loop diuretic, beta-blocker and angiostensin-converting enzyme antagonist. After two months, she was without cardiorespiratory complaints and with improvement in the ejection fraction. Conclusion: Dilated myocardiopathy, despite being a rare manifestation of the use of anti -TNF drugs should be considered in the differential diagnosis of respiratory failure after its use.

Cardiomyopathy, Dilated , Pyoderma Gangrenosum , Heart Failure , Cardiomyopathies , Adalimumab , Antibodies, Monoclonal
Rev. argent. cir. plást ; 26(3): 148-152, 20200900. fig
Article in Spanish | LILACS, BINACIS | ID: biblio-1151847


El pioderma gangrenoso es una patología infrecuente, de origen desconocido, caracterizada por la presencia de úlceras cutáneas de carácter necrótico, de aparición recurrente y rápida progresión. Puede manifestarse en cualquier área anatómica a partir de procesos quirúrgicos o traumáticos, que actúan como disparadores de la reacción autoinmune localizada. En pacientes con cirugía reciente, los signos y síntomas de esta patología se asemejan a una infección posoperatoria. El tratamiento se basa en el uso de corticosteroides e inmunosupresores, containdicando la conducta quirúrgica dado que la misma puede exacerbar la enfermedad. Presentamos un caso de pioderma gangrenoso en mastoplastía posbariátrica con inclusión de prótesis.

Pyoderma gangrenosum is a rare pathology of unknown origin, characterized by the presence of necrotic skin ulcers of recurrent appearance and rapid progression. It can emerge in any anatomic area, after surgeries or traumatic procedures which act as triggers of the localized immune reaction. In patients who have undergone recent surgeries, the signs and symptoms from this pathology resemble a post-operative infection. The treatment consist of corticosteroids and/or immunosuppressants. Surgical procedure is contraindicated since it can exacerbate the disease. We present a case of pyoderma gangrenosum in mastopexy with breast implant insertion post bariatric surgery.

Humans , Female , Adult , Skin Diseases/diagnosis , Pyoderma Gangrenosum/pathology , Breast Implantation , Early Diagnosis
An. bras. dermatol ; 95(2): 203-206, Mar.-Apr. 2020. graf
Article in English | LILACS, ColecionaSUS | ID: biblio-1130837


Abstract Hidradenitis suppurativa is a chronic inflammatory skin disease, which affects 1% of the population, being more common in young, obese and smokers, and mainly affects armpits and groin, with formation of pustules, nodules, abscesses, scars and fistulas. Recently, its association with other autoimmune diseases such as psoriasis, psoriatic arthritis, pyoderma gangrenosum, pyogenic arthritis and ulcerative colitis have been reported. These associated forms are usually resistant to standard treatment, with immunobiologicals as promising therapy. The case of a rare form of association is reported, with only one case previously described in the literature: psoriasis arthritis, pyoderma gangrenosum, acne and hidradenitis suppurativa.

Humans , Female , Young Adult , Arthritis, Psoriatic/pathology , Pyoderma Gangrenosum/pathology , Hidradenitis Suppurativa/pathology , Acne Vulgaris/pathology , Autoimmune Diseases/pathology , Syndrome
Rev. enferm. UFPI ; 9: e9976, mar.-dez. 2020.
Article in Portuguese | LILACS, BDENF | ID: biblio-1371362


Objetivo: relatar o tratamento tópico usado em um caso de Pioderma Gangrenoso em uma mulher de 45 anos que apresentou manifestações clínicas típicas e que obteve boa resposta ao desbridamento cirúrgico, apesar do potencial risco de patergia, bem como apresentar breve revisão da literatura sobre este diagnóstico. Metodologia: estudo do tipo relato de experiência, descritivo, com abordagem qualitativa realizado na Clínica Médica de um Hospital Universitário, no período de abril a junho de 2018. Foi realizado com a autorização da paciente para utilização das suas imagens e informações. Os dados foram obtidos por meio do prontuário, avaliação direta das lesões e registros fotográficos para o acompanhamento da evolução do processo cicatricial. Resultados: constatou-se melhora significativa do processo de cicatrização após desbridamento cirúrgico permitindo, assim, a utilização de curativo com cobertura especial que não seria recomendável sem o procedimento de retirada do tecido necrótico. Conclusão: Os resultados demonstrataram que o desbridamento cirúrgico associado a terapia tópica e sitêmica permitiram uma boa evolução das lesões, embora estudos geralmente recomendem evitar esse desbridamento devido ao risco potencial de patergia.

Objective: To report the topical treatment used in a case of Pyoderma Gangrenosum in a 45-year-old woman who presented typical clinical manifestations and who had a good response to surgical debridement, despite the potential risk of pathergia, as well as to briefly review the literature on this diagnosis. Methodology: it is a descriptive experience report study with a qualitative approach conducted at the Medical Clinic of a University Hospital, from April to June 2018. It was performed with the patient's authorization to use their images and information. Data were obtained through medical records, direct evaluation of the lesions and photographic records to follow the evolution of the healing process. Results: There was a significant improvement in the healing process after surgical debridement, thus allowing the use of a dressing with special coverage that would not be recommended without the necrotic tissue removal procedure. Conclusion: The results showed that surgical debridement associated with topical and systemic therapy allowed a good evolution of the lesions, although studies generally recommend avoiding this debridement due to the potential risk of pathergia.

Colitis, Ulcerative , Pyoderma Gangrenosum , Debridement
Med. interna (Caracas) ; 36(2): 105-110, 2020. ilus
Article in Spanish | LILACS, LIVECS | ID: biblio-1140295


El pioderma gangrenoso es una dermatosis neutrofílica poco común que se presenta como un desorden inflamatorio y ulcerativo de la piel. Más de la mitad de los pacientes que desarrollan esta afección, tienen asociada una enfermedad sistémica subyacente. Caso clínico: paciente femenina de 79 años de edad, previamente sana, quien acude por presentar desde 4 días antes, flictenas violáceas, de contenido serohemático que se decapitan dejando úlcera superficial de fondo eritematoso, y luego profundizaron, no mejoraron con antibióticos por lo que se toma biopsia cutánea que reporta hallazgos compatibles con pioderma gangrenoso; se inició tratamiento con corticoides tópicos y sistémicos evidenciando mejoría de las lesiones. Durante la investigación etiológica se constató descenso de todas las líneas hematológicas y la biopsia de médula ósea mostró síndrome mielodisplásico con displasia unilínea de bajo grado. Discusión: El pioderma gangrenoso de tipo buloso, está comúnmente asociado a enfermedades hematológicas; en pacientes sin alteraciones hematológicas iniciales, debe haber un seguimiento estricto enfocado en la búsqueda de estos trastornos en un lapso de hasta 10 años. Se trata con fármacos inmunosupresores e inmunomoduladores. Conclusiones: ante la presencia de pioderma gangrenoso se debe sospechar síndrome mielodisplásico(AU)

Pyoderma gangrenosum is an uncommon neutrophilic dermatosis that presents as an inflammatory and ulcerative disorder of the skin. More than half of patients with pyoderma gangrenosum develop the disorder in association with an underlying systemic disease. Clinical case: 79-year-old female patient, previously healthy, who consulted for a period of 4 days, purplish skin lesions, which, once decapitated, showed a superficial ulcer that did not improve with broad spectrum antibiotics. The biopsy is taken, which reports findings compatible with pyoderma gangrenosum, so treatment with topical and systemic corticosteroids is started showing improvement of the lesions. During the diagnostic workup, pancytopenia was verified and, after ruling out other etiologies, bone marrow biopsy was performed, which allowed the diagnosis of lower-risk myelodysplastic syndrome with single lineage dysplasia. Discussion: Bullous pyoderma gangrenosum is most commonly seen in patients with hematologic disease; due to the strong association between bullous PG and hematologic disease, patients who present without an associated hematologic disorder should be followed closely for the development of a hematologic disorder. Conclusion: Etiological search for pyoderma gangrenosum allows early diagnosis and timely treatment of the underlying disease(AU)

Humans , Female , Aged , Skin Diseases , Skin Ulcer , Myelodysplastic Syndromes , Pyoderma Gangrenosum , Hematologic Diseases , Biopsy , Adrenal Cortex Hormones/therapeutic use , Immunosuppressive Agents
Article in English | WPRIM | ID: wpr-881513


@#INTRODUCTION: Pyoderma gangrenosum is a rare neutrophilic dermatoses in children of unknown etiology. Its occurrence may be associated with a systemic condition. In most cases, diagnosis is late and treatment is sometimes refractory to conventional therapies especially if the underlying disorder is undetected. CASE REPORT: A 3-year-old Filipino male presented with a one-year history of extensive ulcers over the nape, chest, back and lower extremities which started as a solitary painful pustule over the right gluteal area. Central healing was observed with cribriform scarring. Patient was previously treated as a case of skin infection without success. Significant laboratory findings showed severe anemia, neutrophilia, increased ferritin and increased ESR. Initial hemoglobin electrophoresis showed normal profile with mild microcytic anemia. Bacterial culture and ANA (anti-nuclear antigen) were negative. A course of oral prednisone (1-2mkd) and dapsone (2mkd) were given for a month with no improvement of the lesions. Folic acid alone was started at 5mg per day for anemia. After 2 weeks, lesions significantly improved. Three years later, upon repeat hemoglobin electrophoresis test, patient was diagnosed with alpha-thalassemia trait. Folic acid was given as mainstay therapy. Lesions healed with characteristic cribriform scarring. CONCLUSION: This is a rare case of a Filipino child who initially manifested with pyoderma gangrenosum and eventually diagnosed with alpha-thalassemia trait. Due to serious cosmetic sequelae of pyoderma gangrenosum, it is important to find and treat the underlying systemic disorder to stop progression of this debilitating and disfiguring dermatosis

Pyoderma Gangrenosum , alpha-Thalassemia , Ulcer , beta-Thalassemia , Family
Rev. bras. cir. plást ; 34(4): 567-570, oct.-dec. 2019. ilus
Article in English, Portuguese | LILACS | ID: biblio-1047930


O pioderma gangrenoso (PG) é doença inflamatória da pele, que pode se desenvolver espontaneamente, associado a certas doenças sistêmicas e neoplásicas, ou ao trauma cirúrgico, incluindo os das mamas. Há relatos cada vez mais frequentes, considerando o aumento desse procedimento nos dias atuais. A manifestação clínica das úlceras é característica e deve ser lembrada nas evoluções cicatriciais desfavoráveis com intensa reação inflamatória, perdas teciduais, secreção sanguinolenta e/ ou purulenta, fundo granuloso e bordas elevadas. Relatase o caso de paciente que teve pioderma gangrenoso após mamoplastia redutora. Respondeu ao corticosteroide sistêmico, e vem evoluindo sem recidivas até o momento.

Pyoderma gangrenosum (PG) is an inflammatory disease of the skin that may develop spontaneously. It is associated with certain systemic and neoplastic diseases, including those of the breasts. PG is also associated with surgical trauma. It has been increasingly reported, along with the increase in the incidence of reduction mammoplasty procedures. The clinical manifestation of ulcers is characteristic of PG and it should be considered in cases of poor healing with intense inflammatory reaction, tissue loss, bloody and/ or purulent secretion, granular background, and lesions with high edges. We describe a patient who developed PG after reduction mammoplasty. She has since responded to systemic corticosteroids and has had no relapse to date.

Humans , Female , Middle Aged , History, 21st Century , Postoperative Complications , Skin Diseases , Autoimmune Diseases , Mammaplasty , Pyoderma Gangrenosum , Diagnosis, Differential , Postoperative Complications/surgery , Postoperative Complications/therapy , Skin Diseases/surgery , Skin Diseases/complications , Skin Diseases/therapy , Autoimmune Diseases/diagnosis , Autoimmune Diseases/therapy , Surgical Procedures, Operative , Surgical Procedures, Operative/methods , Mammaplasty/methods , Pyoderma Gangrenosum/surgery , Pyoderma Gangrenosum/complications , Pyoderma Gangrenosum/therapy
An. bras. dermatol ; 94(6): 729-743, Nov.-Dec. 2019. tab, graf
Article in English | LILACS | ID: biblio-1054881


Abstract Pyoderma gangrenosum is a neutrophilic dermatosis characterized by chronic ulcers due to an abnormal immune response. Despite the existence of diagnostic criteria, there is no gold standard for diagnosis or treatment. In Latin America, recognizing and treating pyoderma gangrenosum is even more challenging since skin and soft tissue bacterial and non-bacterial infections are common mimickers. Therefore, this review aims to characterize reported cases of pyoderma gangrenosum in this region in order to assist in the assessment and management of this condition. Brazil, Mexico, Argentina, and Chile are the countries in Latin America that have reported the largest cohort of patients with this disease. The most frequent clinical presentation is the ulcerative form and the most frequently associated conditions are inflammatory bowel diseases, inflammatory arthropaties, and hematologic malignancies. The most common treatment modalities include systemic corticosteroids and cyclosporine. Other reported treatments are methotrexate, dapsone, and cyclophosphamide. Finally, the use of biological therapy is still limited in this region.

Humans , Pyoderma Gangrenosum/diagnosis , Pyoderma Gangrenosum/pathology , Prevalence , Pyoderma Gangrenosum/therapy , Pyoderma Gangrenosum/epidemiology , Diagnosis, Differential , Latin America/epidemiology
An. bras. dermatol ; 94(6): 713-716, Nov.-Dec. 2019. tab, graf
Article in English | LILACS | ID: biblio-1054898


Abstract Hyperzincemia and hypercalprotectinemia is a rare inflammatory disease caused by a mutation in the PSTPIP1 gene, with a dysregulation of calprotectin metabolism. Calprotectin is a zinc-binding protein with antimicrobial properties and pro-inflammatory action. The authors report the case of a 20 year-old girl with cutaneous ulcers comparable with pyoderma gangrenosum, growth failure and chronic anemia, who was given the diagnosis of hyperzincemia and hypercalprotectinemia. Measurement of serum zinc and calprotectin concentrations are indicated in these cases.

Humans , Female , Young Adult , Pyoderma Gangrenosum/pathology , Metal Metabolism, Inborn Errors/pathology , Zinc/blood , Prednisolone/therapeutic use , Treatment Outcome , Cyclosporine/therapeutic use , Leukocyte L1 Antigen Complex/blood , Dermatologic Agents/therapeutic use , Glucocorticoids/therapeutic use , Metal Metabolism, Inborn Errors/drug therapy