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The recurrent intertriginous rash

Published:January 05, 2018DOI:https://doi.org/10.1016/j.ejim.2017.12.016

      Keywords

      1. Case description

      A 51 years old lady presented to the dermatology department for recurrent painful, pruritic and malodourous erosive plaques in the intertriginous folds and scalp since the age of twelve years. Prior treatments included multiple courses of topical antifungals and antibiotics. Six of her siblings and nephews suffered from similar lesions. Physical examination revealed symmetrical extensive erythematous, macerated plaques with erosions over the scalp, axillae, groin and inframammary folds (Fig. 1, Fig. 2), sparing the oral mucosa. A skin biopsy was done over the inframammary folds and scalp. Histopathology features include intraepidermal or suprabasal acantholysis, described as “dilapidated brick wall” (Fig. 3, red arrow). Direct immunofluorescence is negative. Tissue culture for fungal and bacteria were negative. What is her diagnosis?
      Fig. 1
      Fig. 1Symmetrical extensive erythematous, macerated plaques with erosions over the inframammary fold. (For interpretation of the references to colour in this figure legend, the reader is referred to the web version of this article.)
      Fig. 2
      Fig. 2Symmetrical extensive erythematous, macerated plaques with erosions over the groin fold. (For interpretation of the references to colour in this figure legend, the reader is referred to the web version of this article.)
      Fig. 3
      Fig. 3Intraepidermal or suprabasal acantholysis, described as “dilapidated brick wall” (red arrow) (Hematoxylin and eosin stain, original magnification ×100). (For interpretation of the references to colour in this figure legend, the reader is referred to the web version of this article.)

      2. Diagnosis

      Hailey-hailey Disease (HHD), also known as familial benign chronic pemphigus, is a rare chronic blistering disorder with autosomal dominant inheritance. It is first described by brothers Hailey in 1939. It is caused by genetic mutation at ATP2C1 gene at 3q22.1 [
      • Dobson-Stone C.
      • Fairclough R.
      • Dunne E.
      • Brown J.
      • Dissanayake M.
      • Munro C.S.
      • et al.
      Hailey-Hailey disease: molecular and clinical characterization of novel mutations in the ATP2C1 gene.
      ]. The condition has a relapsing-remitting course exacerbated by heat, friction, moisture and UV radiation. The lesions are chiefly in the area that exposed to friction, such as the neck, groin and axilla. Atypical sites of involvement include the scalp, mouth, esophagus and vulva. Asymptomatic longitudinal white lines at the fingernails are occasionally seen [
      • Dobson-Stone C.
      • Fairclough R.
      • Dunne E.
      • Brown J.
      • Dissanayake M.
      • Munro C.S.
      • et al.
      Hailey-Hailey disease: molecular and clinical characterization of novel mutations in the ATP2C1 gene.
      ]. The age of onset is around 30–50 years old. Poor recognition of this disease causes a delay in diagnosis [
      • Benmously-Mlika R.
      • Bchetnia M.
      • Deghais S.
      • Ben Brick S.A.
      • Charfeddine C.
      • Debbiche A.
      • et al.
      Hailey-Hailey disease in Tunisia.
      ]. The diagnosis is clinched by performing a skin biopsy and a positive family history of similar complain. Recurrent fungal, bacteria or viral infection is not uncommon. Treatments of HHD are based on individual case reports or case series due to its rarity. Treatment involves controlling inflammation and treating secondary infection to induce symptomatic remission. In severe diseases, oral dapsone and corticosteroid had been reported to be beneficial. Carbon dioxide laser might be of benefit in recalcitrant cases [
      • Benmously-Mlika R.
      • Bchetnia M.
      • Deghais S.
      • Ben Brick S.A.
      • Charfeddine C.
      • Debbiche A.
      • et al.
      Hailey-Hailey disease in Tunisia.
      ,
      • Pretel-Irazabal M.
      • Lera-Imbuluzqueta J.M.
      • España-Alonso A.
      Carbon dioxide laser treatment in Hailey-Hailey disease: a series of 8 patients.
      ]. Since the introduction of medium potency topical corticosteroid, our patient has responded and is currently in clinical remission.

      Disclaimer

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      Source(s) of support

      None.

      Statement of financial or other relationships that might lead to a conflict of interest

      The authors hereby certify that, to the best of our knowledge, the work which is reported on in said manuscript has not received financial support from any pharmaceutical company or other commercial source and neither us nor any first degree relatives have any special financial interest in the subject matter discussed in said manuscript.

      References

        • Dobson-Stone C.
        • Fairclough R.
        • Dunne E.
        • Brown J.
        • Dissanayake M.
        • Munro C.S.
        • et al.
        Hailey-Hailey disease: molecular and clinical characterization of novel mutations in the ATP2C1 gene.
        J Invest Dermatol. 2002; 118: 338-343
        • Benmously-Mlika R.
        • Bchetnia M.
        • Deghais S.
        • Ben Brick S.A.
        • Charfeddine C.
        • Debbiche A.
        • et al.
        Hailey-Hailey disease in Tunisia.
        Int J Dermatol. 2010; 49: 396-401
        • Pretel-Irazabal M.
        • Lera-Imbuluzqueta J.M.
        • España-Alonso A.
        Carbon dioxide laser treatment in Hailey-Hailey disease: a series of 8 patients.
        Actas Dermosifiliogr. 2013; 104: 325-333