Amicrobial Pustulosis of the Folds Associated with Autoimmune Disorders: Systemic Lupus Erythematosus Case Series and First Report on the Association with Autoimmune Hepatitis

Abstract

Amicrobial pustulosis of the folds (APF) associated with autoimmune disorders is an infrequent entity characterized by the recurrent appearance of follicular and nonfollicular sterile pustules in the context of autoimmune disease. Most reports on APF suggest systemic lupus erythematosus (SLE) as the major immunological associated disorder but the association with autoimmune hepatitis (AH) has not been previously documented. We describe the clinical and histological characteristics of 5 patients with APF: 4 with SLE and 1 with AH. As APF is an exclusion diagnosis, in order to establish an opportune diagnosis and treatment, physicians should be aware of patients with any autoimmune disease who develop a pustular dermatosis for which cultures and stains are negative. We propose the inclusion of anti-liver kidney microsome antibodies in the minor criteria for APF diagnosis.

Amicrobial pustulosis of the folds: Report of 4 cases

“…Our patients also have a broad age distribution, ranging from 23 to 65 years. Consistent with the female predominance reported in the literature, 3 of our 4 patients with APF are women.…”

“…APF was initially reported by Crickx et al at the 1991 French Society of Dermatology. In previous reports, this disease was most often diagnosed in persons between 16 and 51 years of age, with most younger than 40 years . Our patients also have a broad age distribution, ranging from 23 to 65 years.…”

“…Amicrobial pustulosis of the folds (APF) is a rare disorder characterized by aseptic pustules involving skin folds, which is often associated with autoimmune disease and female sex . Since its initial description in 1991, approximately 40 cases are found in the literature . Márquez‐Balbás et al proposed mandatory criteria including pustulosis affecting 1 or more major folds, histologic findings of intraepidermal spongiform pustulosis with neutrophil infiltrate in the dermis, and aseptic pustules.…”

“…Their minor criteria include the association with 1 or more autoimmune disorders, such as antinuclear antibody (ANA) titers of 1:160 or more, and the presence of additional autoantibodies . The differential diagnosis of extensive aseptic pustulosis is broad, including pustular psoriasis, subcorneal pustular dermatosis, acute generalized exanthematous pustulosis, pemphigus foliaceus, immunoglobulin A pemphigus, pemphigus vegetans and pyoderma vegetans . Therefore, clinicopathologic correlation is critical for the diagnosis of APF.…”

Neutrophilic dermatosis

“…Inflammatory bowel diseases are the systemic diseases most frequently encountered in association with pyoderma gangrenosum, especially in its ulcerative or pustular forms, seen in up to 41% of pyoderma gangrenosum cases [5 & ]. It is an uncommon spongiform pustulosis characterized by relapsing follicular and nonfollicular sterile pustules primarily in the skin folds and periorificial regions, specifically encountered in the context of autoimmune disease, most commonly SLE [27]. Amicrobial pustulosis of the folds (APF) may also be regarded as neutrophilic dermatosis.…”