- Email: [email protected]
A Case of Pyodermatitis-Pyostomatitis Vegetans
CASE REPORT
A Case of Pyodermatitis-Pyostomatitis Vegetans Hongmei Wang, MD, Shufang Qiao, MD, Xiujun Zhang, MD and Chun Liu, MD
Abstract: 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. Histology revealed eosinophilic inflammation with microabscesses and pseudoepitheliomatous hyperplasia, but she was negative on direct immunofluorescence for IgA, IgG and C3. She was diagnosed with PD-PSV and treated with infusions of 20% human albumin (100 mL) for 5 days, followed by methylprednisolone (40 mg/d), with remission of lesions observed after 1 month. The differential diagnosis of PD-PSV and pemphigus vegetans is discussed. Key Indexing Terms: Pyodermatitis-pyostomatitis vegetans; Inflammatory bowel disease; Pemphigus vegetans. [Am J Med Sci 2013;345(2):168–171.]
T
CASE REPORT
he report was approved by the research ethics committee of Tianjin Changzhen Hospital, Tianjin, China. A 42-year-old woman diagnosed with ulcerative colitis (UC) 8 years earlier was referred to our department in August 2011 for insidious onset of pustular and vesicular lesions in her oral cavity for 11-month duration. Five months before referral, she began to manifest verrucous and pyogenic lesions on her scalp, neck, axillae, inguinal areas, umbilicus and trunk; these lesions, and those in her oral cavity, had since shown progression, including the formation of vegetating plaques. The lesions were painful and of an annular grouping, as well as being intensively erythematous. On examination, she had a temperature of 36.9°C to 38°C. She had experienced marked general fatigue and swelling in her lower extremities. Her oral mucous membranes were reddish and covered with multiple tiny coalescent pustules creating the typical appearance of “snail tracks” (Figure 1A). Skin examination revealed multiple agglomerated pustules forming red inflammatory plaques on her chest and umbilicus. Large erythematous, indurated, vegetating, erosive and exudative plaques were also seen on her axillae, inguinal areas, umbilicus, scalp and neck (Figure 1B–E). We also observed marked bilateral upper and lower eyelid swelling with lid margin pustules. Histopathologic examination of a biopsy specimen taken from a vegetating plaque on her neck revealed pseudoepitheliomatous hyperplasia and dense eosinophilic inflammation with microabscesses in her dermal papillae and epidermis. The dermis showed dense inflammatory infiltrates composed of eosinophils, neutrophils and lymphocytes (Figure 2A and B). She was negative for IgA, IgG and C3 on direct immunofluorescence testing. Based on these clinical and pathological findings, From the Department of Dermatology, Tianjin Changzhen Hospital, Tianjin, China. Submitted July 10, 2012; accepted in revised form July 30, 2012. The authors report no conflicts of interest. Correspondence: Hongmei Wang, MD, Department of Dermatology, Tianjin Changzhen Hospital, Tianjin 300120, China (E-mail: [email protected]).
168
the patient was diagnosed with pyodermatitis-pyostomatitis vegetans (PD-PSV). Laboratory investigations revealed abnormalities on routine blood tests, including white blood cell (14.48 3 109/L), leukocyte (7.65 3 109/L) and platelet (823 3 109/L) counts, peripheral eosinophilia (2.0 3 109/L), eosinophilic ratio (13.84%) and hemoglobin (101 g/L). Liver function tests also showed abnormalities in total protein (54 g/L) and albumin (28.2 g/L) concentrations and in A/G ratio (1.06). Her erythrocyte sedimentation rate was elevated (45 mm in the first hour). Histology revealed eosinophilic inflammation with microabscesses and pseudoepitheliomatous hyperplasia. She was treated for 5 days with infusions of 20% human albumin (100 mL/d), followed by systemic administration of methylprednisolone (40 mg/d). Improvements were noted 1 week later, including reductions in swelling in her lower extremities. Corticosteroid treatment was slowly titrated to a maintenance dosage of 8 mg/d of prednisone for about 4 months, resulting in significant improvements in her skin and oral cavity lesions (Figure 3A–C). The vegetating plaques resolved with postinflammatory hyperpigmentation and milium. On follow-up, the patient has shown no evidence of recurrence.
DISCUSSION In 1898, 5 patients with oral pustular and cutaneous vegetating lesions were described as having “pyodermite vegetans,” with 2 of these patients presenting with vegetating cutaneous lesions associated with pustular lesions in the oral mucosa.1 In 1949, the term “pyostomatitis vegetans” was introduced to describe 3 patients with isolated oral changes, with 2 of these patients eventually developing cutaneous vegetating lesions with histopathological features similar to those of the oral lesions.2 Most reports support the notion that pyodermatitis vegetans and pyostomatitis vegetans are virtually the same disease. A study of 5 patients with pyodermatitis found that all 5 had UC, suggesting an association between PD-PSV and inflammatory bowel disease (IBD).3 A later study attempted to differentiate pyostomatitis vegetans from pemphigus vegetans–like lesions on the skin, showing that the former was immunofluorescently negative for IgA, IgG and C3 but positive for concomitant IBD, suggesting that PD-PSV was a separate disease entity.4 PD-PSV generally appears at the end of the third decade of life, with women outnumbering men 3:1.5 Table 1 summarizes findings in all patients with PD-PSV described from 2001 to 2012. Clinically, the oral and cutaneous lesions in PD-PSV are unique to this disease and distinctive. Oral manifestations may precede, coincide with or follow cutaneous lesions. Mucosal lesions are characterized by multiple, small friable pustules on an erythematous base, producing a classic “snail tracks” pattern, as observed in our patient. Pain may occur with erosions. Any mucosal surface can be involved, including vaginal, nasal and ocular mucous membranes. Ocular involvement, as in our patient, is very uncommon. Cutaneous manifestations, including the involvement of vesiculopustular, exudative and vegetating plaques, are frequent, occurring in 58% of patients.5 Eruptions are asymmetric and affect mainly the axillae, genital
The American Journal of the Medical Sciences
Volume 345, Number 2, February 2013
A Case of Pyodermatitis-Pyostomatitis Vegetans
FIGURE 1. (A) Photograph of the oral mucous membranes of our patient. The oral mucous membranes were reddish in color and covered with multiple tiny coalescent pustules creating the typical appearance of “snail tracks.” (B) Photograph showing large erythematous, indurated, vegetating, erosive and exudative plaques in the axillae, umbilicus areas, scalp and neck. (C) Photograph showing large erythematous, indurated, vegetating, erosive and exudative plaques in the axillae and surrounding areas. (D) Photograph showing large erythematous, indurated, vegetating, erosive and exudative plaques in the area of the umbilicus. (E) Photograph showing large erythematous, indurated, vegetating, erosive and exudative plaques in the umbilicus and inguinal areas.
areas and scalp. The face, abdomen, umbilicus, trunk and distal extremities are less frequently involved.11 Our patient presented with the characteristic mucocutaneous involvement in typical areas. Our patient differs from previously described patients in having marked general fatigue and hyperphlogosis. In 2009, her liver function was normal, as were tests of her feces and occult blood (++, colloidal gold). On routine blood tests, only her hemoglobin concentration (83 g/L) and platelet count (479 3 109/L) were abnormal. Because of chronic diarrhea caused by UC, the patient was very weak, suggesting that UC may have caused PD-PSV in this patient. Histologically, PD-PSV is characterized by pseudoepitheliomatous hyperplasia with intraepithelial microabscesses and eosinophilic spongiosis. Acantholysis and suprabasilar clefts
have also been observed, as well as dense inflammatory perivascular infiltrates, consisting of neutrophils, eosinophils, lymphocytes and plasmacytes in the dermis.16 Both direct and indirect immunofluorescence assays are negative. Our patient showed similar histopathological findings, whereas other patients have shown nonspecific positive findings, including deposits of C3, IgG and IgA along the basement membrane zone or in the intercellular spaces of the epidermis.16 Blood workup is usually normal, except for elevations in eosinophil counts. Peripheral eosinophilia has been observed in 90% of patients with PD-PSV, making it diagnostic for this condition.5 Cultures for fungi, bacteria and viruses are generally negative. Our patient had been diagnosed with UC 8 years earlier. PD-PSV is closely associated with IBD, with the latter occurring in 70% of patients with PD-PSV.5 PD-PSV is associated more
FIGURE 2. Histologic examination of a biopsy sample showing eosinophilic inflammation with microabscesses and pseudoepitheliomatous hyperplasia (A and B).
Ó 2012 Lippincott Williams & Wilkins
169
Wang et al
FIGURE 3. Photographs showing that the vegetating plaque resolved with postinflammatory hyperpigmentation and milium (A–C).
frequently with UC (53%) than with Crohn’s disease (11%), and approximately 26% of patients with PD-PSV present with some kind of liver dysfunction, such as sclerosing cholangitis, chronic hepatitis or pericolangitis.5 PD-PSV may therefore constitute a hypersensitivity reaction associated with IBD.17–19 Usually, the clinical course of PD-PSV is parallel to that of IBD, and there have been a few reports describing the regression of mucocutaneous lesions after the resolution of an UC
flare or after colectomy.12 Skin lesions, however, may precede gastrointestinal symptoms in approximately 15% of patients, indicating the need for regular and careful gastroenterological monitoring of these patients.4,5,20 Treatment of PD-PSV consists of the application of topical steroids and administration of tacrolimus, dapsone,azathioprine, cyclosporine, isotretinoin, infliximab and/or methotrexate.21 Treatment of IBD with sulfasalazine or mesalazine or colectomy
TABLE 1. Studies of patients with PD-PSV reported from 2001 to 2012 Study and Age Associated Oral Direct Indirect year (yr) Sex disease lesion Cutaneous lesion immunofluorescence immunofluorescence 20015 20015 20016
23 57 32
F F M
No No No
Yes Yes No
20035
22
F
No
Yes
20035
57
F
No
Yes
20047
33
F
UC
Yes
20058
29
M
UC
Yes
20059
19
M
Yes
200810
37
M
200911
47
M
UC/sclerosing cholangitis UC/sclerosing cholangitis/ colon cancer No
Yes
200911 201012
24 51
F F
No UC
Yes Yes
201113
35
M
201114
50
F
UC
Yes
201115
47
F
No
No
Ulcerous pancolitis
Yes
Yes
Treatment
Scalp/vulva-groin Vulva/perianal/limbs Back/feet/heel/ axillae/groin/ extremities Scalp/vulva/groin/ inner thighs Toe/finger/vulva/ groin/perineum Trunk/vulva/eyelid/ scalp
Negative Negative Negative
Negative Negative Not done
Prednisolone Prednisolone Prednisolone, cyclosporine A
Negative
Negative
Prednisone
Negative
Not done
Prednisone
Positive
Positive
Eyelids/groin/thigh/ suprapubic area Scalp/neck/upper trunk/axillae Philtrum/nose wing/ cheek/eyelid/ occipital region
Negative
Negative
Prednisone, dapsone, colchicine Prednisone
Not done
Not done
Isotretinoin
Negative
Not done
Total colectomy, topical tacrolimus
Negative
Not done
Methylprednisolone
Negative Negative
Not done Negative
Genital mucosa/ trunk/ extremities/scalp Eyelid
Positive (IgA)
Positive (weak)
Negative
Not done
Axillae/vulva/ inguinal area/hypogastric area
Negative
Negative
Methylprednisolone Cyclosporine, azathioprine, prednisone Prednisone, mesalazine, mercaptopurine Azathioprine, mesalazine, dapsone Prednisone
Perianal area/low abdomen Scalp Anus/face/neck
UC, ulcerative colitis.
170
Volume 345, Number 2, February 2013
A Case of Pyodermatitis-Pyostomatitis Vegetans
may be effective in treating PD-PSV.10,12 Our patient was sensitive to treatment with methylprednisolone. Nutrition supporting therapy may also be effective. Because PD-PSV and pemphigus vegetans show similar clinical and histopathologic manifestations, there is a need to differentiate between these 2 conditions. A diagnosis of PD-PSV is based on negative direct immunofluorescence assays and the presence of associated intestinal disorders. Other types of dermatitis presenting with vesiculopustular eruptions include bullous pemphigoid, dermatitis herpetiformis, acne conglobata, herpes simplex, erythema multiforme, epidermolysis bullosa acquisita, bullous or atypical pustular granulomatous drug eruptions, Bechet’s disease, the late stages of syphilis, tuberculosis cutis verrucosa, deep fungal infection and halogenodermas.9 The presence of skin and oral pustules, the typical histology and negative immunofluorescence, the peripheral eosinophilia, and the association with IBD narrow the differential diagnosis to PD-PSV. In summary, PD-PSV is a disease with distinctive clinical, histopathologic and immunofluorescence characteristics. PD-PSV is associated with IBD, suggesting that PD-PSV may be a cutaneous marker for IBD. ACKNOWLEDGMENT The authors thank Dr. Ying Guo (Ackerman Academy of Dermotopathology, United States) for diagnosis to this case. REFERENCES 1. Hallopeau H. “Pyodermite vegetante”, ihre Beziehungen zur Dermatitis Herpetiformis und dem Pemphigus Vegetans. Arch Dermatol Syph 1898;43:289–306.
7. Ahn BK, Kim SC. Pyodermatitis-pyostomatitis vegetans with circulating autoantibodies to bullous pemphigoid antigen 230. J Am Acad Dermatol 2004;50:785–8. 8. Leibovitch I, Ooi C, Huilgol SC, et al. Pyodermatitis-pyostomatitis vegetans of the eyelids case report and review of the literature. Ophthalmology 2005;112:1809–13. 9. Konstantopoulou M, O’Dwyer EM, Steele JC, et al. Pyodermatitispyostomatitis vegetans complicated by methicillin-resistant Staphylococcus aureus infection. Clin Exp Dermatol 2005;30:666–8. 10. Yasuda M, Amano H, Nagai Y, et al. Pyodermatitis-pyostomatitis vegetans associated with ulcerative colitis: successful treatment with total colectomy and topical tacrolimus. Dermatology 2008;217:146–8. 11. Ko HC, Jung DS, Jwa SW, et al. Two cases of pyodermatitispyostomatitis vegetans. J Dermatol 2009;36:293–7. 12. Kitayama A, Misago N, Okawa T, et al. Pyodermatitis-pyostomatitis vegetans after subtotal colectomy for ulcerative colitis. J Dermatol 2010;37:714–7. 13. Abellaneda C, Mascaro JM Jr, Vazquez MG, et al. All that glitters is not pemphigus: pyodermatitis-pyostomatitis vegetans misdiagnosed as IgA pemphigus for 8 years. Am J Dermatopathol 2011;33:e1–6. 14. Moloney G, Dolman PJ. Eyelid deformities from pyodermatitis pyostomatitis vegetans. Clin Experiment Ophthalmol 2011;39:910–1. 15. Matias Fde A, Rosa DJ, Carvalho MT, et al. Pyodermatitis-pyostomatitis vegetans: case report and review of medical literature. An Bras Dermatol 2011;86:S137–40. 16. Thornhill MH, Zakrzewska JM, Gilkes JJ. Pyostomatitis vegetans: report of three cases and review of the literature. J Oral Pathol Med 1992;21:128–33.
2. McCarthy FP. Pyostomatitis vegetans; report of three cases. Arch Derm Syphilol 1949;60:750–64.
17. Healy CM, Farthing PM, Williams DM, et al. Pyostomatitis vegetans and associated systemic disease. A review and two case reports. Oral Surg Oral Med Oral Pathol 1994;78:323–8.
3. Brunsting LA, Underwood LJ. Pyoderma vegetans in association with chronic ulcerative colitis. Arch Derm Syphilol 1949;60:161–72.
18. Naish JM, Batchvarov BD, Lawoyin VL. A case of ulcerative colitis and pyostomatitis vegetans in an African. Gut 1970;11:38–40.
4. Storwick GS, Prihoda MB, Fulton RJ, et al. Pyodermatitis-pyostomatitis vegetans: a specific marker for inflammatory bowel disease. J Am Acad Dermatol 1994;31:336–41.
19. Philpot HC, Elewski BE, Banwell JG, et al. Pyostomatitis vegetans and primary sclerosing cholangitis: markers of inflammatory bowel disease. Gastroenterology 1992;103:668–74.
5. Nigen S, Poulin Y, Rochette L, et al. Pyodermatitis-pyostomatitis vegetans: two cases and a review of the literature. J Cutan Med Surg 2003;7:250–5.
20. Soriano ML, Martinez N, Grilli R, et al. Pyodermatitis-pyostomatitis vegetans: report of a case and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1999;87:322–6.
6. Brinkmeier T, Frosch PJ. Pyodermatitis-pyostomatitis vegetans: a clinical course of two decades with response to cyclosporine and low-dose prednisolone. Acta Derm Venereol 2001;81:134–6.
21. Bens G, Laharie D, Beylot-Barry M, et al. Successful treatment with infliximab and methotrexate of pyostomatitis vegetans associated with Crohn’s disease. Br J Dermatol 2003;149:181–4.
Ó 2012 Lippincott Williams & Wilkins
171
A Case of Pyodermatitis-Pyostomatitis Vegetans Hongmei Wang, MD, Shufang Qiao, MD, Xiujun Zhang, MD and Chun Liu, MD
Abstract: 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. Histology revealed eosinophilic inflammation with microabscesses and pseudoepitheliomatous hyperplasia, but she was negative on direct immunofluorescence for IgA, IgG and C3. She was diagnosed with PD-PSV and treated with infusions of 20% human albumin (100 mL) for 5 days, followed by methylprednisolone (40 mg/d), with remission of lesions observed after 1 month. The differential diagnosis of PD-PSV and pemphigus vegetans is discussed. Key Indexing Terms: Pyodermatitis-pyostomatitis vegetans; Inflammatory bowel disease; Pemphigus vegetans. [Am J Med Sci 2013;345(2):168–171.]
T
CASE REPORT
he report was approved by the research ethics committee of Tianjin Changzhen Hospital, Tianjin, China. A 42-year-old woman diagnosed with ulcerative colitis (UC) 8 years earlier was referred to our department in August 2011 for insidious onset of pustular and vesicular lesions in her oral cavity for 11-month duration. Five months before referral, she began to manifest verrucous and pyogenic lesions on her scalp, neck, axillae, inguinal areas, umbilicus and trunk; these lesions, and those in her oral cavity, had since shown progression, including the formation of vegetating plaques. The lesions were painful and of an annular grouping, as well as being intensively erythematous. On examination, she had a temperature of 36.9°C to 38°C. She had experienced marked general fatigue and swelling in her lower extremities. Her oral mucous membranes were reddish and covered with multiple tiny coalescent pustules creating the typical appearance of “snail tracks” (Figure 1A). Skin examination revealed multiple agglomerated pustules forming red inflammatory plaques on her chest and umbilicus. Large erythematous, indurated, vegetating, erosive and exudative plaques were also seen on her axillae, inguinal areas, umbilicus, scalp and neck (Figure 1B–E). We also observed marked bilateral upper and lower eyelid swelling with lid margin pustules. Histopathologic examination of a biopsy specimen taken from a vegetating plaque on her neck revealed pseudoepitheliomatous hyperplasia and dense eosinophilic inflammation with microabscesses in her dermal papillae and epidermis. The dermis showed dense inflammatory infiltrates composed of eosinophils, neutrophils and lymphocytes (Figure 2A and B). She was negative for IgA, IgG and C3 on direct immunofluorescence testing. Based on these clinical and pathological findings, From the Department of Dermatology, Tianjin Changzhen Hospital, Tianjin, China. Submitted July 10, 2012; accepted in revised form July 30, 2012. The authors report no conflicts of interest. Correspondence: Hongmei Wang, MD, Department of Dermatology, Tianjin Changzhen Hospital, Tianjin 300120, China (E-mail: [email protected]).
168
the patient was diagnosed with pyodermatitis-pyostomatitis vegetans (PD-PSV). Laboratory investigations revealed abnormalities on routine blood tests, including white blood cell (14.48 3 109/L), leukocyte (7.65 3 109/L) and platelet (823 3 109/L) counts, peripheral eosinophilia (2.0 3 109/L), eosinophilic ratio (13.84%) and hemoglobin (101 g/L). Liver function tests also showed abnormalities in total protein (54 g/L) and albumin (28.2 g/L) concentrations and in A/G ratio (1.06). Her erythrocyte sedimentation rate was elevated (45 mm in the first hour). Histology revealed eosinophilic inflammation with microabscesses and pseudoepitheliomatous hyperplasia. She was treated for 5 days with infusions of 20% human albumin (100 mL/d), followed by systemic administration of methylprednisolone (40 mg/d). Improvements were noted 1 week later, including reductions in swelling in her lower extremities. Corticosteroid treatment was slowly titrated to a maintenance dosage of 8 mg/d of prednisone for about 4 months, resulting in significant improvements in her skin and oral cavity lesions (Figure 3A–C). The vegetating plaques resolved with postinflammatory hyperpigmentation and milium. On follow-up, the patient has shown no evidence of recurrence.
DISCUSSION In 1898, 5 patients with oral pustular and cutaneous vegetating lesions were described as having “pyodermite vegetans,” with 2 of these patients presenting with vegetating cutaneous lesions associated with pustular lesions in the oral mucosa.1 In 1949, the term “pyostomatitis vegetans” was introduced to describe 3 patients with isolated oral changes, with 2 of these patients eventually developing cutaneous vegetating lesions with histopathological features similar to those of the oral lesions.2 Most reports support the notion that pyodermatitis vegetans and pyostomatitis vegetans are virtually the same disease. A study of 5 patients with pyodermatitis found that all 5 had UC, suggesting an association between PD-PSV and inflammatory bowel disease (IBD).3 A later study attempted to differentiate pyostomatitis vegetans from pemphigus vegetans–like lesions on the skin, showing that the former was immunofluorescently negative for IgA, IgG and C3 but positive for concomitant IBD, suggesting that PD-PSV was a separate disease entity.4 PD-PSV generally appears at the end of the third decade of life, with women outnumbering men 3:1.5 Table 1 summarizes findings in all patients with PD-PSV described from 2001 to 2012. Clinically, the oral and cutaneous lesions in PD-PSV are unique to this disease and distinctive. Oral manifestations may precede, coincide with or follow cutaneous lesions. Mucosal lesions are characterized by multiple, small friable pustules on an erythematous base, producing a classic “snail tracks” pattern, as observed in our patient. Pain may occur with erosions. Any mucosal surface can be involved, including vaginal, nasal and ocular mucous membranes. Ocular involvement, as in our patient, is very uncommon. Cutaneous manifestations, including the involvement of vesiculopustular, exudative and vegetating plaques, are frequent, occurring in 58% of patients.5 Eruptions are asymmetric and affect mainly the axillae, genital
The American Journal of the Medical Sciences
Volume 345, Number 2, February 2013
A Case of Pyodermatitis-Pyostomatitis Vegetans
FIGURE 1. (A) Photograph of the oral mucous membranes of our patient. The oral mucous membranes were reddish in color and covered with multiple tiny coalescent pustules creating the typical appearance of “snail tracks.” (B) Photograph showing large erythematous, indurated, vegetating, erosive and exudative plaques in the axillae, umbilicus areas, scalp and neck. (C) Photograph showing large erythematous, indurated, vegetating, erosive and exudative plaques in the axillae and surrounding areas. (D) Photograph showing large erythematous, indurated, vegetating, erosive and exudative plaques in the area of the umbilicus. (E) Photograph showing large erythematous, indurated, vegetating, erosive and exudative plaques in the umbilicus and inguinal areas.
areas and scalp. The face, abdomen, umbilicus, trunk and distal extremities are less frequently involved.11 Our patient presented with the characteristic mucocutaneous involvement in typical areas. Our patient differs from previously described patients in having marked general fatigue and hyperphlogosis. In 2009, her liver function was normal, as were tests of her feces and occult blood (++, colloidal gold). On routine blood tests, only her hemoglobin concentration (83 g/L) and platelet count (479 3 109/L) were abnormal. Because of chronic diarrhea caused by UC, the patient was very weak, suggesting that UC may have caused PD-PSV in this patient. Histologically, PD-PSV is characterized by pseudoepitheliomatous hyperplasia with intraepithelial microabscesses and eosinophilic spongiosis. Acantholysis and suprabasilar clefts
have also been observed, as well as dense inflammatory perivascular infiltrates, consisting of neutrophils, eosinophils, lymphocytes and plasmacytes in the dermis.16 Both direct and indirect immunofluorescence assays are negative. Our patient showed similar histopathological findings, whereas other patients have shown nonspecific positive findings, including deposits of C3, IgG and IgA along the basement membrane zone or in the intercellular spaces of the epidermis.16 Blood workup is usually normal, except for elevations in eosinophil counts. Peripheral eosinophilia has been observed in 90% of patients with PD-PSV, making it diagnostic for this condition.5 Cultures for fungi, bacteria and viruses are generally negative. Our patient had been diagnosed with UC 8 years earlier. PD-PSV is closely associated with IBD, with the latter occurring in 70% of patients with PD-PSV.5 PD-PSV is associated more
FIGURE 2. Histologic examination of a biopsy sample showing eosinophilic inflammation with microabscesses and pseudoepitheliomatous hyperplasia (A and B).
Ó 2012 Lippincott Williams & Wilkins
169
Wang et al
FIGURE 3. Photographs showing that the vegetating plaque resolved with postinflammatory hyperpigmentation and milium (A–C).
frequently with UC (53%) than with Crohn’s disease (11%), and approximately 26% of patients with PD-PSV present with some kind of liver dysfunction, such as sclerosing cholangitis, chronic hepatitis or pericolangitis.5 PD-PSV may therefore constitute a hypersensitivity reaction associated with IBD.17–19 Usually, the clinical course of PD-PSV is parallel to that of IBD, and there have been a few reports describing the regression of mucocutaneous lesions after the resolution of an UC
flare or after colectomy.12 Skin lesions, however, may precede gastrointestinal symptoms in approximately 15% of patients, indicating the need for regular and careful gastroenterological monitoring of these patients.4,5,20 Treatment of PD-PSV consists of the application of topical steroids and administration of tacrolimus, dapsone,azathioprine, cyclosporine, isotretinoin, infliximab and/or methotrexate.21 Treatment of IBD with sulfasalazine or mesalazine or colectomy
TABLE 1. Studies of patients with PD-PSV reported from 2001 to 2012 Study and Age Associated Oral Direct Indirect year (yr) Sex disease lesion Cutaneous lesion immunofluorescence immunofluorescence 20015 20015 20016
23 57 32
F F M
No No No
Yes Yes No
20035
22
F
No
Yes
20035
57
F
No
Yes
20047
33
F
UC
Yes
20058
29
M
UC
Yes
20059
19
M
Yes
200810
37
M
200911
47
M
UC/sclerosing cholangitis UC/sclerosing cholangitis/ colon cancer No
Yes
200911 201012
24 51
F F
No UC
Yes Yes
201113
35
M
201114
50
F
UC
Yes
201115
47
F
No
No
Ulcerous pancolitis
Yes
Yes
Treatment
Scalp/vulva-groin Vulva/perianal/limbs Back/feet/heel/ axillae/groin/ extremities Scalp/vulva/groin/ inner thighs Toe/finger/vulva/ groin/perineum Trunk/vulva/eyelid/ scalp
Negative Negative Negative
Negative Negative Not done
Prednisolone Prednisolone Prednisolone, cyclosporine A
Negative
Negative
Prednisone
Negative
Not done
Prednisone
Positive
Positive
Eyelids/groin/thigh/ suprapubic area Scalp/neck/upper trunk/axillae Philtrum/nose wing/ cheek/eyelid/ occipital region
Negative
Negative
Prednisone, dapsone, colchicine Prednisone
Not done
Not done
Isotretinoin
Negative
Not done
Total colectomy, topical tacrolimus
Negative
Not done
Methylprednisolone
Negative Negative
Not done Negative
Genital mucosa/ trunk/ extremities/scalp Eyelid
Positive (IgA)
Positive (weak)
Negative
Not done
Axillae/vulva/ inguinal area/hypogastric area
Negative
Negative
Methylprednisolone Cyclosporine, azathioprine, prednisone Prednisone, mesalazine, mercaptopurine Azathioprine, mesalazine, dapsone Prednisone
Perianal area/low abdomen Scalp Anus/face/neck
UC, ulcerative colitis.
170
Volume 345, Number 2, February 2013
A Case of Pyodermatitis-Pyostomatitis Vegetans
may be effective in treating PD-PSV.10,12 Our patient was sensitive to treatment with methylprednisolone. Nutrition supporting therapy may also be effective. Because PD-PSV and pemphigus vegetans show similar clinical and histopathologic manifestations, there is a need to differentiate between these 2 conditions. A diagnosis of PD-PSV is based on negative direct immunofluorescence assays and the presence of associated intestinal disorders. Other types of dermatitis presenting with vesiculopustular eruptions include bullous pemphigoid, dermatitis herpetiformis, acne conglobata, herpes simplex, erythema multiforme, epidermolysis bullosa acquisita, bullous or atypical pustular granulomatous drug eruptions, Bechet’s disease, the late stages of syphilis, tuberculosis cutis verrucosa, deep fungal infection and halogenodermas.9 The presence of skin and oral pustules, the typical histology and negative immunofluorescence, the peripheral eosinophilia, and the association with IBD narrow the differential diagnosis to PD-PSV. In summary, PD-PSV is a disease with distinctive clinical, histopathologic and immunofluorescence characteristics. PD-PSV is associated with IBD, suggesting that PD-PSV may be a cutaneous marker for IBD. ACKNOWLEDGMENT The authors thank Dr. Ying Guo (Ackerman Academy of Dermotopathology, United States) for diagnosis to this case. REFERENCES 1. Hallopeau H. “Pyodermite vegetante”, ihre Beziehungen zur Dermatitis Herpetiformis und dem Pemphigus Vegetans. Arch Dermatol Syph 1898;43:289–306.
7. Ahn BK, Kim SC. Pyodermatitis-pyostomatitis vegetans with circulating autoantibodies to bullous pemphigoid antigen 230. J Am Acad Dermatol 2004;50:785–8. 8. Leibovitch I, Ooi C, Huilgol SC, et al. Pyodermatitis-pyostomatitis vegetans of the eyelids case report and review of the literature. Ophthalmology 2005;112:1809–13. 9. Konstantopoulou M, O’Dwyer EM, Steele JC, et al. Pyodermatitispyostomatitis vegetans complicated by methicillin-resistant Staphylococcus aureus infection. Clin Exp Dermatol 2005;30:666–8. 10. Yasuda M, Amano H, Nagai Y, et al. Pyodermatitis-pyostomatitis vegetans associated with ulcerative colitis: successful treatment with total colectomy and topical tacrolimus. Dermatology 2008;217:146–8. 11. Ko HC, Jung DS, Jwa SW, et al. Two cases of pyodermatitispyostomatitis vegetans. J Dermatol 2009;36:293–7. 12. Kitayama A, Misago N, Okawa T, et al. Pyodermatitis-pyostomatitis vegetans after subtotal colectomy for ulcerative colitis. J Dermatol 2010;37:714–7. 13. Abellaneda C, Mascaro JM Jr, Vazquez MG, et al. All that glitters is not pemphigus: pyodermatitis-pyostomatitis vegetans misdiagnosed as IgA pemphigus for 8 years. Am J Dermatopathol 2011;33:e1–6. 14. Moloney G, Dolman PJ. Eyelid deformities from pyodermatitis pyostomatitis vegetans. Clin Experiment Ophthalmol 2011;39:910–1. 15. Matias Fde A, Rosa DJ, Carvalho MT, et al. Pyodermatitis-pyostomatitis vegetans: case report and review of medical literature. An Bras Dermatol 2011;86:S137–40. 16. Thornhill MH, Zakrzewska JM, Gilkes JJ. Pyostomatitis vegetans: report of three cases and review of the literature. J Oral Pathol Med 1992;21:128–33.
2. McCarthy FP. Pyostomatitis vegetans; report of three cases. Arch Derm Syphilol 1949;60:750–64.
17. Healy CM, Farthing PM, Williams DM, et al. Pyostomatitis vegetans and associated systemic disease. A review and two case reports. Oral Surg Oral Med Oral Pathol 1994;78:323–8.
3. Brunsting LA, Underwood LJ. Pyoderma vegetans in association with chronic ulcerative colitis. Arch Derm Syphilol 1949;60:161–72.
18. Naish JM, Batchvarov BD, Lawoyin VL. A case of ulcerative colitis and pyostomatitis vegetans in an African. Gut 1970;11:38–40.
4. Storwick GS, Prihoda MB, Fulton RJ, et al. Pyodermatitis-pyostomatitis vegetans: a specific marker for inflammatory bowel disease. J Am Acad Dermatol 1994;31:336–41.
19. Philpot HC, Elewski BE, Banwell JG, et al. Pyostomatitis vegetans and primary sclerosing cholangitis: markers of inflammatory bowel disease. Gastroenterology 1992;103:668–74.
5. Nigen S, Poulin Y, Rochette L, et al. Pyodermatitis-pyostomatitis vegetans: two cases and a review of the literature. J Cutan Med Surg 2003;7:250–5.
20. Soriano ML, Martinez N, Grilli R, et al. Pyodermatitis-pyostomatitis vegetans: report of a case and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1999;87:322–6.
6. Brinkmeier T, Frosch PJ. Pyodermatitis-pyostomatitis vegetans: a clinical course of two decades with response to cyclosporine and low-dose prednisolone. Acta Derm Venereol 2001;81:134–6.
21. Bens G, Laharie D, Beylot-Barry M, et al. Successful treatment with infliximab and methotrexate of pyostomatitis vegetans associated with Crohn’s disease. Br J Dermatol 2003;149:181–4.
Ó 2012 Lippincott Williams & Wilkins
171