Indian Journal of Dermatology
  Publication of IADVL, WB
  Official organ of AADV
Indexed with Science Citation Index (E) , Web of Science and PubMed
 
Users online: 132  
Home About  Editorial Board  Current Issue Archives Online Early Coming Soon Guidelines Subscriptions  e-Alerts    Login  
    Small font sizeDefault font sizeIncrease font size Print this page Email this page


 
Table of Contents 
CORRESPONDENCE
Year : 2020  |  Volume : 65  |  Issue : 4  |  Page : 323-324
Amicrobial pustulosis of the folds: Report of three new cases


1 Section of Dermatology and Venereology, Department of Medicine, University of Verona, Verona, Italy
2 Section of Pathology, Department of Diagnostics and Public Health, University of Verona, Verona, Italy

Date of Web Publication11-Jun-2020

Correspondence Address:
Francesco Bellinato
From the Section of Dermatology and Venereology, Department of Medicine, University of Verona, Verona
Italy
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijd.IJD_120_19

Rights and Permissions



How to cite this article:
Bellinato F, Maurelli M, Colato C, Girolomoni G. Amicrobial pustulosis of the folds: Report of three new cases. Indian J Dermatol 2020;65:323-4

How to cite this URL:
Bellinato F, Maurelli M, Colato C, Girolomoni G. Amicrobial pustulosis of the folds: Report of three new cases. Indian J Dermatol [serial online] 2020 [cited 2020 Jul 8];65:323-4. Available from: http://www.e-ijd.org/text.asp?2020/65/4/323/286387




Sir,

Amicrobial pustulosis of the folds (APF) is a rare, chronic and relapsing disease belonging to neutrophil dermatosis. APF involves major and small folds and is associated with autoimmune disorders and/or immunological abnormalities.[1],[2],[3] Herein, we report the clinical, pathological, and laboratory findings of three new cases of APF.

The first case was a 46-year-old woman who presented with a 10-year history of recurrent pustular eruption on an erythematous base on the neck, axillae, submammary folds, lip commissures, pubis, and scalp [Figure 1]a and [Figure 1]b. Histological examination showed hyperplasia with focal parakeratosis, mild exocytosis of neutrophils, spongiform pustules in the epidermis, and a scant, perivascular neutrophilic infiltrate in the superficial and mid dermis. The patient was treated with dapsone 75 mg/day and topical mometasone furoate, with marked improvement of the lesions after 12 weeks. The remission was obtained with a 7-day cycle of prednisone 0.5 mg/kg/day.
Figure 1: (a) Crusted and oozing erosive patches involving nape of neck. (b) Involvement of external auditory canal. (c) Erythematous plaques with nonfollicular pustules involving submammary folds. (d) Involvement of the axillary area. (e) Hyperplastic epidermis with neutrophil exocytosis forming subcorneal aggregates. Mixed neutrophilic and lymphocytic infiltrate in the upper dermis with perivascular and interstitial distribution. Mild edema of the dermal papillae associated with superficial dermal infiltrate of lymphocytes, with perivascular and interstitial distribution (H and E, ×400)

Click here to view


The second case was a 40-year-old woman who presented with a chronic pustular eruption with oozing and crusts of 4-month duration involving the inguinal folds, perineum, scalp, ears, and nose orifices. Histopathology showed parakeratosis, intraepidermal vesicles and pustules of neutrophils, and an infiltrate of lymphocytes and neutrophils in the superficial dermis. The patient was treated with oral corticosteroids (1 mg/kg/day) and oral clarithromycin 500 mg/bid for 5 days, with rapid improvement. In the following 3 years, the patient underwent four exacerbations, always responding to 0.5 mg/kg/day prednisone alone for 7 days.

The third case was a 44-year-old Hispanic woman affected by systemic lupus erythematosus (SLE) since the age of 18 years and with a 3-year history of recurrent erosions and nonfollicular pustules involving the intergluteal and submammary folds, axillae, genital area, retroauricular groove, and occipital region of the scalp [Figure 1]c and d]. Histopathology showed hyperplastic epidermis with neutrophil exocytosis forming subcorneal aggregates. The upper dermis showed a mixed neutrophilic and lymphocytic infiltrate with perivascular and interstitial distribution [Figure 1]e. The patient was treated with oral corticosteroids (1 mg/kg/day) and topical mometasone furoate with clinical benefit after 2 weeks and total remission after 10 weeks. At 2 years of follow-up, the patient maintained complete remission.

To date, 69 cases of APF have been reported in the literature, with a 90% female prevalence. Marzano et al. coined the term APF and suggested diagnostic criteria.[1] The list of differential diagnoses covers most subcorneal pustulosis as well as bacterial and candidal intertrigo.[2] All our three cases showed an involvement of the major and minor folds, scalp, and genital area with erythematous plaques, erosions, and few nonfollicular pustules at the edge. Several cultures for bacteria and fungi collected from unopened pustules before antibiotic treatment were consistently negative. One patient had a previous diagnosis of SLE and one had autoimmune thyroiditis.[4] ANA positivity was found in all our cases at a titer >1/640 with a homogeneous or speckled pattern. Histopathology showed spongiform subcorneal pustules and inflammatory dermal neutrophilic infiltrate with scattered lymphocytes.[1] Given the rarity of APF, there are no standard therapeutic regimens. Systemic corticosteroids (0.5 mg/kg/day) represent the most effective treatment.[1] Other therapies include dapsone 50–100 mg/day, cyclosporine 3–3.5 mg/kg/day, and steroid-sparing adjunctive therapies, such as colchicine 1 mg/day, hydroxychloroquine 400 mg/day, and methotrexate 15 mg/week.[5] The clinical course of APF is chronic-relapsing, and in our series, only one patient showed no relapse.

Our cases confirmed that APF has a chronic course, but benign clinical behavior. Given the rarity of APF, there is no consensus on best treatment. We found a good response with low-dose systemic corticosteroids.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Marzano AV, Ramoni S, Caputo R. Amicrobial pustulosis of the folds. Dermatology 2008;216:305-11.  Back to cited text no. 1
    
2.
Schissler C, Velter C, Lipsker D. Amicrobial pustulosis of the folds: Where have we gone 25 years after its original description? Ann Dermatol Venereol 2017;144:169-75.  Back to cited text no. 2
    
3.
Wang MZ, Camilleri MJ, Guo R, Wieland CN. Amicrobial pustulosis of the folds: Report of 4 cases. J Cutan Pathol 2017;44:367-72.  Back to cited text no. 3
    
4.
Lòpez-Navarro N, Alcaide A, Gallego E, Herrera-Acosta E, Gallardo M, Bosch RJ, et al. Amicrobial pustulosis of the folds associated with Hashimoto's thyroiditis. Clin Exp Dermatol 2009;34:e561-3.  Back to cited text no. 4
    
5.
Kerl K, Masouyé I, Lesavre P, Saurat JH, Borradori L. A case of amicrobial pustulosis of the folds associated with neutrophilic gastrointestinal involvement in systemic lupus erythematosus. Dermatology 2005;211:356-9.  Back to cited text no. 5
    


    Figures

  [Figure 1]



 

Top
Print this article  Email this article
 
 
  Search
 
  
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
    Article in PDF (696 KB)
    Citation Manager
    Access Statistics
    Reader Comments
    Email Alert *
    Add to My List *
* Registration required (free)  


    References
    Article Figures

 Article Access Statistics
    Viewed95    
    Printed1    
    Emailed0    
    PDF Downloaded3    
    Comments [Add]    

Recommend this journal