Clinical Review & Education
JAMA Dermatology Clinicopathological Challenge
Widespread Ulcerated and Vegetative Plaques With an Unusual Rhinophymatous Nose Caroline Champagne, MBChB, MRCP; Emily Davies, MRCP; Sophie Grabczynska, FRCP
A tattoo artist in his 40s presented with a 5-month history of painful, oozing lesions over his trunk and limbs. A year earlier, he was a victim in an armed robbery and was shot in the right thigh. He reported that he has had problems with widespread abscess formation since then. His only medical history is of long-standing fulminant rhinophyma. He is a smoker but drinks minimal alcohol. On examination, he had multiple ulcerative and vegetative plaques with several pustules on his scalp, arms, groin, legs, and back (Figure, A). He also had a marked rhinophymatous nose, which was inflamed and ulcerated superiorly (Figure, B). There was no mucosal involvement and no lymphadenopathy, and he was apyrexial.
Blood tests revealed a microcytic anemia and features of chronic inflammation or infection, but all other tests, including a vasculitis screen, human immunodeficiency virus (HIV) test, and chest radiograph, had normal results. Serum bromide and iodide levels were normal. Skin swab samples cultured Staphylococcus aureus, and tissue culture grew coliforms and enterococci after a prolonged period of enrichment. A shave biopsy sample from the nose (Figure, C and D) revealed histological features similar to those of punch biopsy samples from the vegetative plaques. Direct and indirect immunofluorescence had negative results. What is your diagnosis?
A
B
C
D
Figure. A, Annular vegetative plaque with surrounding pustular lesions. B, Ulcerated and inflamed rhinophymatous nose. C, Hematoxylin-eosin (H&E) staining shows pseudoepitheliomatous hyperplasia with microabscesses (original magnification ×40). D, Neutrophil- and eosinophil-rich microabscesses (H&E, original magnification ×100).
jamadermatology.com
JAMA Dermatology July 2014 Volume 150, Number 7
Copyright 2014 American Medical Association. All rights reserved.
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773
Clinical Review & Education JAMA Dermatology Clinicopathological Challenge
Diagnosis Pyoderma vegetans
Microscopic Findings and Clinical Course Histopathological examination revealed pseudoepitheliomatous hyperplasia, together with neutrophil- and eosinophil-rich microabscesses. There were no granulomas, and Gram and periodic acid– Schiff staining had negative results. He was treated with oral minocycline hydrochloride 100 mg twice a day, a weaning course of oral prednisolone starting at 30 mg, and topical clobetasol propionate ointment. He improved but not substantially, so oral dapsone was added and titrated up to 100 mg daily. The lesions responded well, leaving postinflammatory hyperpigmentation. However, the rhinophymatous nose (which we do not believe is true sebaceous hyperplasia) is proving difficult to control and often flares, responding only to additional oral steroids.
Discussion Pyoderma vegetans (PV) is a chronic inflammatory vegetating disorder first described by Hallopeau in 1898.1 Several other synonyms have been used including pseudoepithelioma of Azua, pyodermatitis vegetans (usually combined with pyostomatitis), hyperinflammatory proliferative pyoderma, and blastomycosislike pyoderma.2,3 Clinically, the exudative vegetative plaques with pustules favor the face, scalp, and intertriginous sites and usually heal with postinflammatory hyperpigmentation.3 The etiology is unknown, but it is believed to be due to an excessive inflammatory response to factors such as bacterial infection in predisposed immunosuppressed patients. However, it has also been reported in healthy patients without immune deficiency.2-4 Staphylococcus aureus is the most commonly isolated pathogen.4 Pyoderma vegetans can also occur at sites of tattoos and foreign-body reactions and can follow minor trauma ARTICLE INFORMATION Author Affiliations: Department of Dermatology, Amersham Hospital, Buckinghamshire National Health Service Trust, Amersham, England (Champagne, Davies, Grabczynska); Department of Dermatology, Churchill Hospital, Oxford University Hospitals National Health Service Trust, Oxford, England (Champagne). Corresponding Author: Caroline Champagne, MBChB, MRCP, Department of Dermatology, Churchill Hospital, Old Rd, Headington, Oxford, Oxfordshire OX3 7LE, England ([email protected]). Section Editor: Molly A. Hinshaw, MD; Assistant Section Editors: Soon Bahrami, MD; Nicole Fett,
774
to the skin.3 It has been linked with several underlying diseases such as chronic myeloid leukemia, HIV infection, chronic malnutrition, and alcoholism, and the pyodermatitis-pyostomatitis vegetans variant is often associated with inflammatory bowel disease.3,4 The cutaneous lesions may be the first manifestations of an underlying disease.4 The diagnosis is made on the basis of characteristic histological findings and excluding other diseases that can cause ulcerovegetative lesions such as pemphigus vegetans, pyoderma gangrenosum (PG) vegetans, Wegener granulomatosis, halogenoderma, and deep fungal or mycobacterial infections.3 In 1979, Su et al5 proposed 6 diagnostic criteria: (1) large verrucous plaques with pustules and an elevated border, (2) pseudoepitheliomatous hyperplasia with abscesses histologically, (3) growth of a least 1 pathogenic bacterial species from tissue culture, (4) culture negative for deep fungi and mycobacteria, (5) negative results on fungal serologic tests, and (6) normal bromide and iodide levels in the blood. There is no standard management and often the response to treatment is poor, but there are reports of favorable results with administration of culture-directed antibiotics together with other therapies including oral and intralesional corticosteroids, dapsone, acitretin, cyclosporine, disodium cromoglycate, etanercept, laser ablation, and excision.3,4 Our patient meets many of the criteria for PV; however, the vegetative variant of PG, also known as superficial granulomatous pyoderma, remains the main differential diagnosis.6 Pyoderma gangrenosum vegetans tends to respond to less aggressive antiinflammatory therapy, which is not the case in our patient. In addition, no classical ulcerative PG lesions were seen, there is no sign of clinical pathergy or cribriform scarring, and evidence of bacterial colonization was repeatedly found, suggesting that PV is the diagnosis rather than PG vegetans. However, some authors believe that PV and PG are in fact variants of the same disease.2
MD, MSCE; Anna K. Haemel, MD; Arni K. Kristjansson, MD; Lori D. Prok, MD. Published Online: May 28, 2014. doi:10.1001/jamadermatol.2013.9845. Conflict of Interest Disclosures: None reported. REFERENCES 1. Hallopeau H. Pyodermite vegetante, ihre Beziehungen zur Dermatitis Herpetiformis and dem Pemphigus vegetans. Arch f Dermatol u Syph. 1898;43:289-306. 2. Bianchi L, Carrozzo AM, Orlandi A, Campione E, Hagman JH, Chimenti S. Pyoderma vegetans and ulcerative colitis. Br J Dermatol. 2001;144(6): 1224-1227.
3. Adişen E, Tezel F, Gürer MA. Pyoderma vegetans: a case for discussion. Acta Derm Venereol. 2009;89(2):186-188. 4. Cerullo L, Zussman J, Young L. An unusual presentation of blastomycosislike pyoderma (pyoderma vegetans) and a review of the literature. Cutis. 2009;84(4):201-204. 5. Su WP, Duncan SC, Perry HO. Blastomycosis-like pyoderma. Arch Dermatol. 1979;115(2):170-173. 6. Wilson-Jones E, Winkelmann RK. Superficial granulomatous pyoderma: a localized vegetative form of pyoderma gangrenosum. J Am Acad Dermatol. 1988;18(3):511-521.
JAMA Dermatology July 2014 Volume 150, Number 7
Copyright 2014 American Medical Association. All rights reserved.
Downloaded From: http://archderm.jamanetwork.com/ by a University Of Connecticut Health Center User on 05/11/2015
jamadermatology.com
JAMA Dermatology Clinicopathological Challenge
Widespread Ulcerated and Vegetative Plaques With an Unusual Rhinophymatous Nose Caroline Champagne, MBChB, MRCP; Emily Davies, MRCP; Sophie Grabczynska, FRCP
A tattoo artist in his 40s presented with a 5-month history of painful, oozing lesions over his trunk and limbs. A year earlier, he was a victim in an armed robbery and was shot in the right thigh. He reported that he has had problems with widespread abscess formation since then. His only medical history is of long-standing fulminant rhinophyma. He is a smoker but drinks minimal alcohol. On examination, he had multiple ulcerative and vegetative plaques with several pustules on his scalp, arms, groin, legs, and back (Figure, A). He also had a marked rhinophymatous nose, which was inflamed and ulcerated superiorly (Figure, B). There was no mucosal involvement and no lymphadenopathy, and he was apyrexial.
Blood tests revealed a microcytic anemia and features of chronic inflammation or infection, but all other tests, including a vasculitis screen, human immunodeficiency virus (HIV) test, and chest radiograph, had normal results. Serum bromide and iodide levels were normal. Skin swab samples cultured Staphylococcus aureus, and tissue culture grew coliforms and enterococci after a prolonged period of enrichment. A shave biopsy sample from the nose (Figure, C and D) revealed histological features similar to those of punch biopsy samples from the vegetative plaques. Direct and indirect immunofluorescence had negative results. What is your diagnosis?
A
B
C
D
Figure. A, Annular vegetative plaque with surrounding pustular lesions. B, Ulcerated and inflamed rhinophymatous nose. C, Hematoxylin-eosin (H&E) staining shows pseudoepitheliomatous hyperplasia with microabscesses (original magnification ×40). D, Neutrophil- and eosinophil-rich microabscesses (H&E, original magnification ×100).
jamadermatology.com
JAMA Dermatology July 2014 Volume 150, Number 7
Copyright 2014 American Medical Association. All rights reserved.
Downloaded From: http://archderm.jamanetwork.com/ by a University Of Connecticut Health Center User on 05/11/2015
773
Clinical Review & Education JAMA Dermatology Clinicopathological Challenge
Diagnosis Pyoderma vegetans
Microscopic Findings and Clinical Course Histopathological examination revealed pseudoepitheliomatous hyperplasia, together with neutrophil- and eosinophil-rich microabscesses. There were no granulomas, and Gram and periodic acid– Schiff staining had negative results. He was treated with oral minocycline hydrochloride 100 mg twice a day, a weaning course of oral prednisolone starting at 30 mg, and topical clobetasol propionate ointment. He improved but not substantially, so oral dapsone was added and titrated up to 100 mg daily. The lesions responded well, leaving postinflammatory hyperpigmentation. However, the rhinophymatous nose (which we do not believe is true sebaceous hyperplasia) is proving difficult to control and often flares, responding only to additional oral steroids.
Discussion Pyoderma vegetans (PV) is a chronic inflammatory vegetating disorder first described by Hallopeau in 1898.1 Several other synonyms have been used including pseudoepithelioma of Azua, pyodermatitis vegetans (usually combined with pyostomatitis), hyperinflammatory proliferative pyoderma, and blastomycosislike pyoderma.2,3 Clinically, the exudative vegetative plaques with pustules favor the face, scalp, and intertriginous sites and usually heal with postinflammatory hyperpigmentation.3 The etiology is unknown, but it is believed to be due to an excessive inflammatory response to factors such as bacterial infection in predisposed immunosuppressed patients. However, it has also been reported in healthy patients without immune deficiency.2-4 Staphylococcus aureus is the most commonly isolated pathogen.4 Pyoderma vegetans can also occur at sites of tattoos and foreign-body reactions and can follow minor trauma ARTICLE INFORMATION Author Affiliations: Department of Dermatology, Amersham Hospital, Buckinghamshire National Health Service Trust, Amersham, England (Champagne, Davies, Grabczynska); Department of Dermatology, Churchill Hospital, Oxford University Hospitals National Health Service Trust, Oxford, England (Champagne). Corresponding Author: Caroline Champagne, MBChB, MRCP, Department of Dermatology, Churchill Hospital, Old Rd, Headington, Oxford, Oxfordshire OX3 7LE, England ([email protected]). Section Editor: Molly A. Hinshaw, MD; Assistant Section Editors: Soon Bahrami, MD; Nicole Fett,
774
to the skin.3 It has been linked with several underlying diseases such as chronic myeloid leukemia, HIV infection, chronic malnutrition, and alcoholism, and the pyodermatitis-pyostomatitis vegetans variant is often associated with inflammatory bowel disease.3,4 The cutaneous lesions may be the first manifestations of an underlying disease.4 The diagnosis is made on the basis of characteristic histological findings and excluding other diseases that can cause ulcerovegetative lesions such as pemphigus vegetans, pyoderma gangrenosum (PG) vegetans, Wegener granulomatosis, halogenoderma, and deep fungal or mycobacterial infections.3 In 1979, Su et al5 proposed 6 diagnostic criteria: (1) large verrucous plaques with pustules and an elevated border, (2) pseudoepitheliomatous hyperplasia with abscesses histologically, (3) growth of a least 1 pathogenic bacterial species from tissue culture, (4) culture negative for deep fungi and mycobacteria, (5) negative results on fungal serologic tests, and (6) normal bromide and iodide levels in the blood. There is no standard management and often the response to treatment is poor, but there are reports of favorable results with administration of culture-directed antibiotics together with other therapies including oral and intralesional corticosteroids, dapsone, acitretin, cyclosporine, disodium cromoglycate, etanercept, laser ablation, and excision.3,4 Our patient meets many of the criteria for PV; however, the vegetative variant of PG, also known as superficial granulomatous pyoderma, remains the main differential diagnosis.6 Pyoderma gangrenosum vegetans tends to respond to less aggressive antiinflammatory therapy, which is not the case in our patient. In addition, no classical ulcerative PG lesions were seen, there is no sign of clinical pathergy or cribriform scarring, and evidence of bacterial colonization was repeatedly found, suggesting that PV is the diagnosis rather than PG vegetans. However, some authors believe that PV and PG are in fact variants of the same disease.2
MD, MSCE; Anna K. Haemel, MD; Arni K. Kristjansson, MD; Lori D. Prok, MD. Published Online: May 28, 2014. doi:10.1001/jamadermatol.2013.9845. Conflict of Interest Disclosures: None reported. REFERENCES 1. Hallopeau H. Pyodermite vegetante, ihre Beziehungen zur Dermatitis Herpetiformis and dem Pemphigus vegetans. Arch f Dermatol u Syph. 1898;43:289-306. 2. Bianchi L, Carrozzo AM, Orlandi A, Campione E, Hagman JH, Chimenti S. Pyoderma vegetans and ulcerative colitis. Br J Dermatol. 2001;144(6): 1224-1227.
3. Adişen E, Tezel F, Gürer MA. Pyoderma vegetans: a case for discussion. Acta Derm Venereol. 2009;89(2):186-188. 4. Cerullo L, Zussman J, Young L. An unusual presentation of blastomycosislike pyoderma (pyoderma vegetans) and a review of the literature. Cutis. 2009;84(4):201-204. 5. Su WP, Duncan SC, Perry HO. Blastomycosis-like pyoderma. Arch Dermatol. 1979;115(2):170-173. 6. Wilson-Jones E, Winkelmann RK. Superficial granulomatous pyoderma: a localized vegetative form of pyoderma gangrenosum. J Am Acad Dermatol. 1988;18(3):511-521.
JAMA Dermatology July 2014 Volume 150, Number 7
Copyright 2014 American Medical Association. All rights reserved.
Downloaded From: http://archderm.jamanetwork.com/ by a University Of Connecticut Health Center User on 05/11/2015
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