Case for diagnosis

Abstract

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. Bowel surveillance is mandatory, since the dermatosis is associated with inflammatory bowel diseases in more than 70% of patients, especially ulcerative colitis.

Spectrum of autoimmune bullous diseases among children in Kuwait

“…PD-PSV is known to be associated with IBD, particularly UC and PSC. [20][21][22][23] Our patient had PSC, UC, and anterior uveitis (AU). The association of AU with IBD has been previously reported.…”

“…PS-PDV is a very rare inflammatory mucocutaneous dermatitis, mostly reported in adults, that is rare in children. [20][21][22][23] Immunopathological findings in PS-PDV suggest it represents IgA or IgA/IgG pemphigus. 20 The presence of immunoreactants in the BMZ, similar to that observed in our patient, has been proposed to result from epitope spreading.…”

Pyostomatitis Vegetans With Orofacial and Vulvar Granulomatosis in a Pediatric Patient

“…9,30 However, PSV is incredibly rare in children and adolescents and can be an early finding of IBD in children. 16,31,32 Our patient has not developed GI involvement since her initial presentation 5 years prior, though another pediatric patient developed symptomatic CD 9 years after onset of OFG. 5 A retrospective review of pediatric OFG without CD met criteria for CD at a median of 3.1 years (range, 0.4-6.9 years).…”

“…28,29 Two recent articles that looked at PSV in pediatric and adolescent populations identified only 9 patients with PSV. 24,30 Only 2 patients (siblings) had documented onset of PSV before 12 years of age, 31 which suggests an underlying genetic predisposition in young children.…”

Pyodermatitis-pyostomatitis vegetans and antibullous pemphigoid antigen 180 autoantibodies: a casual association?

“…The diagnosis of PD‐PSV was made on the basis of the clinical presentation and histopathological findings. Pemphigus vegetans was excluded because of the absence of intercellular deposits of IgG and C3 in the epidermidis . Mucous membrane pemphigoid was excluded on the basis of clinical features (presence of pustules and vegetant erosions), histopathological aspects (epidermal hyperplasia and acantholytic blisters) and direct immunofluorescence (granular C3 deposits).…”