Pyoderma gangrenosum: update and guidance

VIEIRA, LUIZ FELIPE DUARTE FERNANDES; ALMEIDA, CARLOS LACERDA ANDRADE; SOUZA, ALEXANDRE WAGNER DE; RÊGO, JOZELIA; VALE, EVERTON SIVIERO DO; PEGAS, JOSE ROBERTO PEREIRA

doi:10.5935/2177-1235.2022RBCP.671-en

LUIZ FELIPE DUARTE FERNANDES VIEIRA ^**Corresponding author: Luiz Felipe Duarte Fernandes-Vieira Av. Boa Viagem, 296/1004, Pina, Recife, PE, Brazil, Zip Code: 51011-000, E-mail: luizfelipedfv@gmail.com

Analysis and/or interpretation of data

Final approval of the manuscript

Conceptualization

Conception and design of the study

Project Management

Methodology

Writing - Review and Editing

Supervision

Hospital Agamenon Magalhaes, Departamento de Cirurgia Plástica, Recife, PE, Brazil

https://orcid.org/0000-0002-9774-9821

CARLOS LACERDA ANDRADE ALMEIDA

Analysis and/or interpretation of data

Research

Hospital Agamenon Magalhaes, Departamento de Cirurgia Plástica, Recife, PE, Brazil

https://orcid.org/0000-0003-4815-903X

ALEXANDRE WAGNER DE SOUZA

Research

Methodology

Universidade Federal de São Paulo UNIFESP-EPM, Disciplina de Reumatologia, São Paulo, SP, Brazil

https://orcid.org/0000-0001-7681-6215

JOZELIA RÊGO

Analysis and/or interpretation of data

Conception and design of the study

Research

Universidade Federal de Goiás Faculdade de Medicina Serviço de Reumatologia, Goiânia, GO, Brazil

https://orcid.org/0000-0002-8935-1506

EVERTON SIVIERO DO VALE

Conception and design of the study

Investigation

Universidade Federal de Minas Gerais, Departamento de Dermatologia, Belo Horizonte, MG, Brazil

https://orcid.org/0000-0002-9172-3639

JOSE ROBERTO PEREIRA PEGAS

Conception and design of the study

Investigation

Faculdade de Medicina de Jundiaí, Departamento de Dermatologia, Jundiaí, SP, Brazil

https://orcid.org/0000-0002-2541-6008

*Corresponding author: Luiz Felipe Duarte Fernandes-Vieira Av. Boa Viagem, 296/1004, Pina, Recife, PE, Brazil, Zip Code: 51011-000, E-mail: luizfelipedfv@gmail.com

Conflicts of interest: none.

Variant	Clinical presentation	Common locations	Associated systemic
Ulcerative	Inflammatory pustules or nodules that rapidly progress to necrotic ulcers with violaceous undermining edges with surrounding erythema	Trauma sites	Inflammatory bowel disease Hematologic maligna Rheumatoid arthritis Seronegative arthritis Monoclonal gammopathy
Ulcerative		Anterior face of lower limbs
Bullous	Painful blister that can progress to erosion and/or a rapidly evolving ulcer	Face	ex. Acute myeloid leukemia, inflammatory bowel disease
Bullous		Upper and lower limbs	ex. Acute myeloid leukemia, inflammatory bowel disease
Pustular	Pustules with erythematous edges and Symmetrical	Lower members	Inflammatory bowel disease
Pustular	Pustules with erythematous edges and Symmetrical	Trunk	Inflammatory bowel disease
Vegetative	Less painful variant	Trunk	None
	Slow growth
	No purulent
	Single superficial ulcer, non-subminated and less violaceous borders
	Respond quickly to therapy
Peristomal	Papules that evolve into ulcers with subminated borders	Immediately adjacent to the stoma	Enteric malignancy
Peristomal	Difficult to distinguish from other peristomal erosive lesions	Immediately adjacent to the stoma	Connective tissue disease Monoclonal gammopathy
Postoperative	Erythema at the surgical site followed by dehiscence ulcer OR ulcerations that coalesce	Surgical site	Commonly associated with chest and abdomen surgery
Postoperative	Disproportionately increased pain	Surgical site	Commonly associated with chest and abdomen surgery

Groups	Frequency	Illnesses
Inflammatory bowel disease	41.0%	Crohn’s disease
		Ulcerative colitis
		Rheumatoid arthritis
Inflammatory arthropathies	20.5%	Enteropathy
		Arthropathy
		Psoriatic arthritis
		Ankylosing spondylitis
Hematologic neoplasms	5.9%	Arthritis unspecified
		Non-Hodgkin’s lymphoma
		Acute myeloid leukemia
		Chronic myelomonocytic leukemia
Non-malignant hematologic diseases	4.8%	Large granular cell
		lymphocytic leukemia
		Myelofibrosis
		Myelodysplastic syndrome monoclonal gammopathy of undetermined origin
		Polycythemia vera
Solid organ neoplasms	6.5%	--

Autoinflammatory syndromes	Main clinical manifestations	Genes
PAPA syndrome	PG, acne and sterile pyogenic arthritis	PSTPIP1
PASH syndrome	PG, acne and hidradenitis suppurativa	MEFV, NOD2, NLRP3, PSMB8, NCSTN
PAPASH syndrome	Pyogenic arthritis, PG, acne hidradenitis suppurativa	PSTPIP1, IL1RN, MEFV
SAPHO syndrome	Synovitis, acne, pustulosis, hyperostosis and osteitis	PSTPIP2, LPIN2, NOD2
PASS syndrome	Espondiloartrite axial, PG, acne conglobata e hidradenite supurativa	--
PsAPASH syndrome	Psoriatic arthritis, PG, hidradenitis suppurativa and acne	--

Infections
	Viral (chronic herpes simplex, cytomegalovirus)
	Bacterial (ecthyma, gangrenous ecthyma, tuberculosis, atypical mycobacteriosis, necrotizing fasciitis)
	Parasitic (amebiasis, cutaneous leishmaniasis)
	Fungal (sporotrichosis, paracoccidioidomycosis, cryptococcosis, histoplasmosis, aspergillosis)
*Vasculitis and vasculopathies*
	Behcet’s disease
	Cutaneous and systemic vasculitis (leukocytoclastic vasculitis, granulomatosis with polyangiitis, polyarteritis nodosa)
	Livedoid vasculopathy
	Antiphospholipid antibody syndrome
	Collagen diseases (systemic lupus erythematosus, systemic sclerosis)
*Occlusive vascular disease and venous disease*
	Venous ulcer
	Hypertensive ulcer
	Sickle cell disease ulcer
	Peripheral arterial obstructive disease
	Trophic ulcers(neuropathic)
*Neoplasms*
	Cutaneous leucemia
	Cutaneouslymphomas
	Basal cell carcinoma
	Squamous cell carcinomas
*Miscellaneous*
	Factitious dermatitis
	Injuries from injecting illicit drugs
	Halogenoderma
	Loxoscelism
	Calciphylaxis

Major criterion
Ulcer edge biopsy showing neutrophilic infiltrate
Minor criteria
*Histology*
Infection exclusion (special stains and tissue cultures)
*History*
Pathergy (occurrence of ulcers at sites of trauma)
Personal history of inflammatory bowel disease or inflammatory arthritis History of a papule, pustule, or vesicle rapidly progressing to ulceration
Physical examination (or photographic record)
Peripheral erythema, detached edge, hypersensitivity at the site of ulceration, Multiple ulcers (at least one in the anterior region of the leg)
Cribriform or “wrinkled-paper” scar after ulcer resolution
*Treatment*
Reduction in ulcer size after one month of immunosuppressive treatment

[1] *Corresponding author: Luiz Felipe Duarte Fernandes-Vieira Av. Boa Viagem, 296/1004, Pina, Recife, PE, Brazil, Zip Code: 51011-000, E-mail: luizfelipedfv@gmail.com

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Pyoderma gangrenosum: update and guidance

ABSTRACT

Introduction:

Methods:

Results:

Conclusion: