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Amicrobial pustulosis of the folds: Report of 4 cases
Michael Z Wang1, Michael J Camilleri1,2, Ruifeng Guo1
1Department of Dermatology, Mayo Clinic, Rochester, Minnesota.
Abstract:
Amicrobial pustulosis of the folds (APF) is a rare disease characterized by aseptic pustular lesions involving cutaneous folds, typically occurring in the context of an autoimmune disorder. We present 4 patients with APF, focusing on clinical and histopathologic characteristics to improve the recognition of this entity. All 4 patients had intertriginous and extra-intertriginous involvement. Common histopathologic features of skin biopsies in these patients were intracorneal, subcorneal, intraepidermal, perivascular, perifollicular and interstitial neutrophilic inflammation. Pustules overlying adnexal ostium and papillary dermal edema were consistently observed. The pustules were negative for microorganisms on stain testing. In these cases, associated conditions were undifferentiated connective tissue disease, systemic lupus erythematosus, antiphospholipid syndrome, Crohn disease and Hashimoto thyroiditis. The aforementioned clinical and histopathologic characteristics of patients with suspected or diagnosed connective tissue disorders should lead to suspicion for APF.
Insights
Amicrobial pustulosis of the folds (APF) is a rare autoimmune skin condition. Recognizing its distinct clinical and histopathologic features aids in diagnosing this aseptic pustular disease.
Area of Science:
- Dermatology
- Pathology
- Autoimmunology
Background:
- Amicrobial pustulosis of the folds (APF) is a rare condition.
- It presents as aseptic pustular lesions in skin folds.
- APF often occurs with autoimmune disorders.
Observation:
- This study details 4 patients with APF.
- Clinical and histopathologic features were analyzed.
- Both intertriginous and extra-intertriginous areas were involved.
Findings:
- Histopathology revealed neutrophilic inflammation (intracorneal, subcorneal, intraepidermal, perivascular, perifollicular, interstitial).
- Pustules over adnexal ostia and papillary dermal edema were consistent findings.
- Microorganisms were absent in pustule stains.
Implications:
- APF diagnosis requires recognizing specific clinical and histopathologic signs.
- Associated conditions included connective tissue diseases and autoimmune disorders.
- Suspicion of APF is warranted in patients with connective tissue disorders exhibiting these features.
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