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Cutis 2019 Jan;103(1):44-45

Advanced Dermatology Associates, Ltd, Allentown, Pennsylvania, USA.

Necrobiosis lipoidica (NL) is a granulomatous inflammatory skin disease strongly associated with diabetes mellitus (DM). Red-brown papules expanding into plaques with erythematous indurated borders on the lower extremities are characteristic of NL. Diagnosis is made clinically; however, biopsy of lesions confirms the diagnosis. Read More

Postepy Dermatol Alergol 2018 Apr 24;35(2):212-216. Epub 2018 Apr 24.

Chair of Dermatology, Sexually Transmitted Diseases and Immunodermatology, Nicolaus Copernicus University in Torun, Faculty of Medicine in Bydgoszcz, Poland.

BMJ Case Rep 2017 Oct 13;2017. Epub 2017 Oct 13.

Manchester Academic Health Science Centre, The University of Manchester, Manchester, UK.

We describe a case of disfiguring facial pyoderma vegetans in order to highlight the challenges in managing this rare skin condition and review the literature.A 54-year-old woman presented to dermatology clinic with a 3-month history of a left-sided facial lesion, which had been treated as an infected sebaceous cyst. The lesion had dramatically increased in size in the weeks prior to presentation. Read More

Int J Dermatol 2017 12 5;56(12):1457-1459. Epub 2017 May 5.

Department of Dermatology, University of Minnesota Medical School, Minneapolis, MN, USA.

Hautarzt 2016 Dec;67(12):940-947

Abteilung für Dermatologie und Venerologie, Krankenanstalt Rudolfstiftung, Juchgasse 25, 1030, Wien, Österreich.

Background: Over recent decades, both the incidence and prevalence of chronic inflammatory bowel disease have continued to rise in industrialized countries; the disease is frequently associated with extracutaneous involvement and comorbidity.

Objectives: The purpose of this work was to investigate the frequency and specificity of mucocutaneous manifestations in Crohn's disease (CD) and ulcerative colitis (UC).

Materials And Methods: An extensive search in peer-reviewed journals via PubMed was performed; presented is a summary and analysis of various studies and data, including data of patients treated at our department. Read More

Dermatol Online J 2016 Jun 16;22(6). Epub 2016 Jun 16.

Department of Dermatology, Centro Hospitalar do Porto, Oporto, Portugal.

Pyodermatitis vegetans (PV) is a rare inflammatory dermatosis of unknown etiology and difficult diagnosis characterized by vesiculopustular, exudative, and vegetating plaques usually localized in the axillary, genital, and oral region. It bears similarities to pemphigus vegetans but clinical history, histology, and immunofluorescence help confirm the diagnosis. Pyodermatitis vegetans is often associated with an underlying gastrointestinal disorder, especially ulcerative colitis. Read More

J Am Acad Dermatol 2016 Sep 24;75(3):578-584. Epub 2016 Jun 24.

Department of Dermatology, Mayo Clinic, Rochester, Minnesota; Division of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota. Electronic address:

Background: Pyostomatitis vegetans (PSV)-pyodermatitis vegetans (PDV) is a rare inflammatory mucocutaneous disease associated with inflammatory bowel disease.

Objective: We sought to evaluate the clinicopathologic findings of PSV-PDV in a series of 7 patients.

Methods: We conducted a retrospective review of all cases of PSV-PDV at the Mayo Clinic from 1995 to 2014. Read More

Am J Dermatopathol 2016 Feb;38(2):148-50

*Department of Dermatology, Şişli Hamidiye Etfal Training and Research Hospital, Istanbul, Turkey; and †Department of Pathology, Cerrahpasa Faculty of Medicine, Istanbul University, Istanbul, Turkey.

Pyoderma vegetans, a rare disorder of the skin, is considered a highly specific marker for inflammatory bowel disease, especially ulcerative colitis. It is clinically characterized by large verrucous plaques with elevated borders and multiple pustules. Here, the authors report the case of a 33-year-old man who was misdiagnosed as having verrucous carcinoma for 4 years. Read More

Am J Dermatopathol 2015 Sep;37(9):699-700, 730-2

*Georgetown University School of Medicine, Washington, DC; and †Department of Dermatology, Washington Hospital Center, Georgetown University, Washington, DC.

Rom J Morphol Embryol 2015 ;56(2):563-8

Department of Surgery, University of Medicine and Pharmacy of Craiova, Romania;

Pyoderma vegetans is a rare disease characterized by the presence of vegetant exudative, pustular and erythematous vesiculobullous plaque usually located in the inguinal area and axillary fold. Etiology of pyoderma vegetans is unknown but it is often associated with bacterial infections in immunocompromised patients. Main histopathological characteristics of pyoderma crops are pseudoepitheliomatous hyperplasia and subepidermal, intraepidermal neutrophilic or eosinophilic microabscesses. Read More

Iran J Med Sci 2015 Jul;40(4):381-5

Pediatric Congenital Hematologic Disorders Research Center, Mofid Children Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran.

Pyoderma vegetans (PV) is a rare inflammatory disorder characterized by vegetating pustules and plaques affecting the skin and mucosal membranes. It is believed that this entity is mostly associated with inflammatory bowel disease (IBD), chronic malnutrition, human immunodeficiency virus (HIV), malignancies, and other immunocompromised states. Pyoderma vegetans occurs more commonly in young and middle-aged adults. Read More

Middle East J Dig Dis 2015 Apr;7(2):104-6

Professor, Digestive Disease Research Center, Shariati Hospital, Tehran University of Medical Science,Tehran,Iran.

Some dermatologic manifestations are common in ulcerative colitis (UC). Herein, we present a 36-year-old woman with ulcerative colitis and uncommon nasal mucosa pyoderma vegetans. The patient presented to our hospital with symptoms of active colitis and a concomitant 3×4×5 cm dermato-mucosal lesion in her left nasal lumen. Read More

Rev Med Chir Soc Med Nat Iasi 2015 Jan-Mar;119(1):107-11

Pyoderma vegetans is a rare disorder that more commonly affects middle-aged persons, with a male predilection. It is characterized by vegetating lesions that coalescence into a plaque with eroded surface, covered by purulent discharge and crusts. The etiology of this disease is not known with certainty, but it is often associated with bacterial infections in immunocompromised patients. Read More

Br J Dermatol 2015 Mar 5;172(3):811-3. Epub 2015 Feb 5.

Section of Clinical, Allergological and Venereological Dermatology, Department of Medicine, University of Perugia, Perugia, 06156, Italy.

Genet Couns 2013 ;24(2):133-9

Department of Dermatology, Yuzuncu Yil University, Faculty of Medicine, Van, Turkey.

GAPO syndrome is a rare autosomal recessive disease and an acronym composed of growth retardation, alopecia, pseudoanodontia, optic atrophy. Approximately 38 cases have been reported in literature until now. Pyoderma vegetans is a chronic inflammatory disease characterized with vesicopustular, exudative and vegetative lesions usually localized on face, scalp, axilla and genitalia. Read More

Dermatol Online J 2013 Jun 15;19(6):18558. Epub 2013 Jun 15.

Weill Cornell Medical College, New York, NY, USA.

Hidradenitis suppurativa (HS), a pathologic follicular disease, impacts patients' lives profoundly and usually occurs in isolation. The diseases with the strongest association are obesity, depression, and pain. HS is associated with many diseases including acne conglobata (AC), dissecting cellulitis, pilonidal cysts, and obesity. Read More

J Am Acad Dermatol 2013 Feb;68(2):211.e1-33; quiz 244-6

Division of Dermatology, Department of Internal Medicine, Baylor University Medical Center, Dallas, Texas 75246, USA.

The gastrointestinal (GI) and cutaneous organ systems are closely linked. In part I of this continuing medical education article, the intricacies of this relationship were explored as they pertained to hereditary polyposis disorders, hamartomatous disorders, and paraneoplastic disease. Part II focuses on the cutaneous system's links to inflammatory bowel disease and vascular disorders. Read More

Ann Dermatol Venereol 2012 Dec 3;139(12):836-7. Epub 2012 Sep 3.

An Bras Dermatol 2012 Nov-Dec;87(6):929-31

University of Brasilia (Universidade de Brasilia - UnB) - Brasilia (DF), Brazil.

Pyodermatitis-pyostomatitis vegetans is a rare mucocutaneous dermatosis characterized by pustular and vegetating lesions of the skin and oral mucosa. It is considered a highly specific marker for inflammatory bowel diseases. The authors describe a case of pyodermatitis-pyostomatitis vegetans in a pediatric patient who presented marked clinical improvement after beginning treatment with oral corticosteroids, azathioprine, and dapsone. Read More

Am J Med Sci 2013 Feb;345(2):168-71

Department of Dermatology, Tianjin Changzhen Hospital, Tianjin, China.

Pyodermatitis-pyostomatitis vegetans (PD-PSV) is a rare disorder characterized by mucocutaneous involvement and associated with inflammatory bowel disease. A 42-year-old woman with ulcerative colitis who manifested verrucous and pyogenic lesions on her scalp, neck, axillae, inguinal areas, umbilicus, trunk and oral cavity for about 11 months is described. She also experienced general fatigue and swelling in her lower extremities. Read More

J Oral Pathol Med 2012 Sep 5;41(8):584-8. Epub 2012 May 5.

Department of Dermatology, Medical School, University of São Paulo, São Paulo, Brazil.

Pyostomatitis vegetans (PSV) is an intraoral pustular eruption considered by most authors to represent the mucous analogous of cutaneous pyoderma gangrenosum and its vegetating presentations (pyodermatitis vegetans). A strong correlation of PSV with inflammatory bowel disease (IBD) is well documented. The histopathology of PSV lesions usually reveals acanthosis, and neutrophils and/or eosinophils infiltration with intraepithelial or subepithelial abscesses; acantholysis is present in some cases. Read More

Indian J Dermatol 2012 Jan;57(1):71-2

Ann Gastroenterol 2012 ;25(1):21-26

Inflammatory Bowel Disease Unit of the Gastroenterology and Hepatology Department of the University Clinic Hospital of Valencia (Jesus Manuel Santiago, Rosario Antón, Marta Maia Bosca).

Almost one-third of patients with inflammatory bowel disease (IBD) develop skin lesions. Cutaneous disorders associated with IBD may be divided into 5 groups based on the nature of the association: specific manifestations (orofacial and metastatic IBD), reactive disorders (erythema nodosum, pyoderma gangrenosum, pyodermatitis-pyostomatitis vegetans, Sweet's syndrome and cutaneous polyarteritis nodosa), miscellaneous (epidermolysis bullosa acquisita, bullous pemphigoid, linear IgA bullous disease, squamous cell carcinoma-Bowen's disease, hidradenitis suppurativa, secondary amyloidosis and psoriasis), manifestations secondary to malnutrition and malabsorption (zinc, vitamins and iron deficiency), and manifestations secondary to drug therapy (salicylates, immunosupressors, biological agents, antibiotics and steroids). Treatment should be individualized and directed to treating the underlying IBD as well as the specific dermatologic condition. Read More

An Bras Dermatol 2011 Jul-Aug;86(4 Suppl 1):S137-40

Serviço de Dermatologia, Hospital Universitário, Universidade Federal de Juiz de Fora, Juiz de Fora, MG, Brasil.

Pyodermatitis-pyostomatitis vegetans is a rare inflammatory dermatosis of unknown etiology, with a typical mucocutaneous involvement. We report the case of a woman with pustular and vesicular lesions in the axillae, evolving with vegetating plaques and pustules with annular grouping. The disease progressed with vulvar and inguinal involvement as well as involvement of the oral, nasal and ocular mucous membranes. Read More

Acta Dermatovenerol Croat 2011 ;19(3):180-2

Department of Dermatology and Venereology, University Hospital Center Zagreb, School of Medicine University of Zagreb, Croatia.

Pyoderma chronica vegetans is a rare condition clinically characterized by large verrucous plaques with elevated borders and multiple pustules. Although the etiology is unknown, it is generally believed that vegetating reaction is due to an immune system dysfunction or immunosuppressive state. We report on a 72-year-old man with pyoderma chronica of the buttocks. Read More

Clin Exp Ophthalmol 2011 Dec 14;39(9):910-1. Epub 2011 Jun 14.

Case Rep Dermatol 2011 Mar 26;3(1):80-4. Epub 2011 Mar 26.

Department of Dermatology, Ministry of Health Diskapi Yildirim Beyazit Education and Research Hospital, Ankara, Turkey.

Pyoderma vegetans (PV) is a very rare disorder characterized by erythematous, vesiculopustular, exudative, vegetating plaques usually localized in the inguinal and axillary folds. The etiology of PV is not known, but it is often associated with bacterial infections in immunocompromised patients. Major histopathological features in PV are pseudoepitheliomatous hyperplasia and intraepidermal and subepidermal neutrophilic or eosinophilic microabscesses. Read More

Indian J Dermatol 2010 Oct;55(4):379-80

Royal Brisbane Hospital, Brisbane.

A 41-year-old male with known congenital IgA deficiency developed pyoderma vegetans during a subtropical holiday. He responded well to topical treatment with aluminium subacetate. Read More

Dermatol Online J 2011 Mar 15;17(3). Epub 2011 Mar 15.

Department of Dermatology, Hospital of Pistoia, Pistoia, Italy.

Blastomycosis-like pyoderma, also known as pyoderma vegetans, is an uncommon disease characterized by vegetating skin lesions mimicking blastomycosis or warty tuberculosis, often associated with staphylococcal and streptococcal infections. It is more commonly observed in elderly patients with impaired immunological capacity, and is often unresponsive to various therapeutic modalities. We describe a case of BLP that occurred on the palmar aspect of the left hand in an elderly female, affected with recurrent vesicular hand eczema. Read More

Zh Mikrobiol Epidemiol Immunobiol 2010 Jul-Aug(4):31-7

Aim: Assessment of therapeutic effect and immunologic parameters during use of Immunovac vaccine for complex treatment of chronic forms of pyoderma.

Materials And Methods: Ninety-five patients with different clinical forms of chronic pyoderma (furunculosis, hydradenitis, chronic ulcerative and ulcerative-vegetans pyoderma, folliculitis, impetigo etc.) were studied. Read More

Am J Dermatopathol 2011 Feb;33(1):e1-6

Department of Dermatology, Hospital Clínic Barcelona University, Barcelona, Spain.

Pyodermatitis-pyostomatitis vegetans is a rare mucocutaneous dermatosis often associated with gastrointestinal disorders, especially with inflammatory bowel disease. It is clinically characterized by erythematous lesions with multiple pustules and erosions affecting the mucosal surfaces. Cutaneous lesions are characterized by exudative and vegetating plaques affecting frequently the axillae and groins. Read More

J Dermatol 2010 Aug;37(8):714-7

Division of Dermatology, Department of Internal Medicine, Faculty of Medicine, Saga University, Saga, Japan.

A 51-year-old Japanese woman underwent subtotal colectomy for ulcerative colitis 5 years before her first visit to our hospital, and her symptoms stabilized. She presented with a 2-month history of proliferative, pustular lesions on her face, scalp, and gingival and anal mucosae. Histological examination showed features similar to those of pemphigus vegetans. Read More

Cutis 2009 Oct;84(4):201-4

Division of Dermatology, David Geffen School of Medicine, University of California, Los Angeles. 90095-6957, USA.

We report a case of blastomycosislike pyoderma (BLP), also known as pyoderma vegetans, in a 75-year-old woman. The patient initially presented with multiple lesions, some classic and others with highly uncharacteristic morphology. The appearance of unusual purulent hornlike lesions on both cheeks delayed recognition of the disease. Read More

Transplantation 2009 Jun;87(12):1898-9

Int J Dermatol 2009 Jun;48(6):665-6

J Dermatol 2009 May;36(5):293-7

Department of Dermatology, School of Medicine, Pusan National University, Busan 602-739, Korea.

Pyodermatitis-pyostomatitis vegetans (PPV) is a rare chronic pustular and vegetating mucocutaneous dermatosis. The oral lesions present as multiple, friable and yellowish pustules, which form "snail tracts" and rupture easily. The cutaneous lesions begin as crusted erythematous papulopustules that coalesce to form large vegetating plaques, usually in the axillae, genital area and scalp. Read More

Acta Derm Venereol 2009 ;89(2):186-8

Ann Dermatol Venereol 2008 Nov 23;135(11):753-6. Epub 2008 Jul 23.

Service de dermatologie, CHU de Nantes, Hôtel Dieu, 1, place Alexis-Ricordeau, 44093 Nantes cedex 1, France.

Background: Pyodermatitis-pyostomatitis vegetans (PPV) is a rare chronic disorder often associated with inflammatory bowel disease. We report an isolated case involving the oral, labial and nasal mucosa.

Patients And Methods: A 28-year-old man, in good general condition, presented with a 2-year history of painless stomatitis. Read More

Dermatology 2008 4;217(2):146-8. Epub 2008 Jun 4.

Department of Dermatology, Gunma University Graduate School of Medicine, Maebashi, Japan.

Int J Dermatol 2007 Mar;46(3):299-302

Dermatology, Medical University of Warsaw, Warsaw, Poland.

A 58-year-old woman with ulcerative colitis of 5 years' duration was first seen in March 2002 with a 5-month history of widespread erosions on the palate and gingiva, subtle scarring of the conjunctiva, and xerostomia accompanying a flare of ulcerative colitis. No skin lesions were observed. Direct immunofluorescence study performed on the oral mucosa showed a linear distribution of immunoglobulin G (IgG) and immunoglobulin A (IgA) at the dermal-epidermal junction. Read More

Dermatology 2007 ;214(1):77-81

Department of Dermatology, Hospital Clínic de Barcelona, Barcelona University, Barcelona, Spain.

Pyoderma vegetans (PV) is an inflammatory dermatosis, characterized clinically by large exudative vegetating plaques, and histopathologically by epidermal pseudoepitheliomatous hyperplasia and dense inflammatory infiltrates. Although PV is a very rare condition, it is a chronic disorder that may accompany any systemic process that compromises immunity. Treatment is very difficult, and correction of predisposing causes may be useful. Read More

J Postgrad Med 2006 Oct-Dec;52(4):302-3

Department of Gastroenterology, Calicut Medical College, Kozhikode - 673 008, Kerala, India.

Mycoses 2006 Mar;49(2):143-4

Chronic vegetating pyoderma is usually associated with staphylococci and streptococci. A case that started as a therapeutically resistant tinea of the hand caused by Trichophyton mentagrophytes is described. The vegetating skin lesion was associated with low immunoglobulin levels and impaired phagocytosis. Read More

Clin Exp Dermatol 2005 Nov;30(6):666-8

Department of Oral Medicine, Liverpool University Dental Hospital & School of Dentistry, Liverpool, UK.

Pyodermatitis-pyostomatitis vegetans (PPV), a rare disorder of the skin and oral mucosa, is considered a highly specific marker for inflammatory bowel disease, especially ulcerative colitis (UC). Oral lesions (pyostomatitis vegetans) are seen without skin involvement but rarely without gastrointestinal symptoms. Bowel symptoms may be minimal and precede the onset of other lesions by months or years. Read More

Postgrad Med J 2005 Sep;81(959):580-5

Department of Dermatology, A61, Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland, Ohio 44195, USA.

Inflammatory bowel disease (IBD) has many extraintestinal manifestations. Cutaneous manifestations are usually related to the activity of the bowel disease but may have an independent course. Anyone presenting with IBD should be examined for cutaneous manifestations. Read More

Ophthalmology 2005 Oct;112(10):1809-13

Oculoplastic & Orbital Division, Department of Ophthalmology & Visual Sciences, Royal Adelaide Hospital, University of Adelaide, Adelaide, Australia.

Objectives: To present the first reported case of eyelid involvement in pyodermatitis-pyostomatitis vegetans (PDPSV) leading to the diagnosis of ulcerative colitis, and to review the literature.

Design: Interventional case report.

Methods: A 29-year-old man presented with a 4-week history of severe bilateral upper and lower eyelid margin ulceration and pustules unresponsive to topical and systemic broad-spectrum antibiotic treatment. Read More

J Am Acad Dermatol 2004 May;50(5):785-8

Department of Dermatology and Cutaneous Biology Research Institute, Yongdong Severance Hospital, Yonsei University College of Medicine, Seoul, South Korea.

We describe a woman from Korea with pyodermatitis-pyostomatitis vegetans associated with ulcerative colitis. On immunofluorescence examination, she demonstrated in vivo bound and circulating IgG antibasement membrane zone antibodies. The immunoelectron microscopy and immunoblot analysis showed that the antibodies reacted with the bullous pemphigoid antigen 230. Read More

J Urol 2004 Jan;171(1):354

Brady Urological Institute and Department of Dematology, Johns Hopkins Medical Institutions, Baltimore, Maryland 21287, USA.

J Cutan Med Surg 2003 May-Jun;7(3):250-5

Department of Dermatology, Centre Hospitalier Universitaire de Québec, pavillon Hotel-Dieu de Québec, Québec, Québec, Canada.

Background: Pyodermatitis-pyostomatitis vegetans (PD-PSV) is a rare vegetating, pustular, eosinophilic, mucocutaneous dermatosis characterized by mucocutaneous lesions of the genital, axillary, and oral regions, as well as on the scalp.

Objective: We report two patients who were diagnosed with PD-PSV. The published cases of this rare disorder are reviewed. Read More

J Cutan Med Surg 2001 May-Jun;5(3):223-7. Epub 2001 May 17.

Department of Dermatology, New Jersey Medical School, Newark, New Jersey 07103-2714, USA.

Background: Pyoderma vegetans is a rare condition that is clinically characterized by large verrucous plaques with elevated borders and multiple pustules. The etiology of this disorder remains unknown.

Objectives: We describe a 24-year-old woman with rapidly evolving pyoderma vegetans. Read More