Ipoh, Malaysia
The patient, in his 50s, presented with a 10-year history of recurrent pruritic skin eruptions on the groins and axilla. He was treated by his GPs as well as a urologist for "infected tinea cruris" with oral antifungals, IV antibiotics, topical steroids, topical antifungals but the treatments did not help. For the past 3 months, the lesions have worsened. He had no fever or other constitutional symptoms. He had no history of diabetes. His father apparently had similar skin lesions.
Examination showed erythematous plaques with vesiculation and superficial fissures and erosions on the groins extending to the scrotum, inner thighs and intergluteal cleft. The lesions appeared to be bilateral, symmetrical, hyperpigmented and well demarcated. Similar lesions were noted on the axilla. There were no bulla or purpura. There was no oral or nail lesions. KOH exam for hyphae was negative on 3 occasions.
Initial provisional diagnosis was that of tinea incognito but KOH exam was repeatedly negative. Hailey-Hailey disease a.k.a. familial benign chronic pemphigus was considered in the differential diagnosis.
A skin biopsy with DIF was done. H&E shows focal and central lesion of suprabasal and intraepithelial clefts containing basophilic cells with large nuclei and a paranuclear halo. There is a presence of basal keratinocytes attached to basement membrane forming a characteristic tombstone, or ‘‘dilapidated brick wall’’ appearance. Corps ronda cells are not prominent. The stratum corneum shows parakeratosis and hyperkeratosis with focal neutrophilic infiltrates. The upper dermis shows perivascular infiltrate of lymphocytes, histiocytes and eosinophils.
Immunofluorescence studies show IgG, IgA , IgM, C3 were negative.
Interpretation: Acantholytic epithelial lesion consistent with familial benign pemphigus.
The management of this patient may be challenging. General measures such as wearing lightweight and loose clothing would be helpful. Avoiding friction on the affected areas, use of mild antiseptic solutions and reduction in sweating are also advisable. In terms of specific treatment, we plan to start with topical calcineurin inhibitors and oral antibiotics such as doxycycline 100mg bd. Other possible options include low dose naltrexone, methotrexate, dapsone, azathioprine, etanercept, apremilast, botox and laser treatment. Low dose naltrexone appeared to be very effective in some of the cases but may have variable responses according to the dose.
References
1. Imene Ben Lagha, Kurt Ashack, Amor Khachemoune. Hailey Disease: An Update Review with a Focus on Treatment Data.American Journal of Clinical Dermatology Oct 2019. https://doi.org/10.1007/s40257-019-00477-z
2. Hohl D. Darier disease and Hailey–Hailey disease. In: Bolognia J, Jorizzo J, Schaffer J, editors. Philadelphia: Elsevier Inc; 2012. p. 887–97.
3. Severine Cao, Evelyn Lilly, Steven T. Chen. Variable Response to Naltrexone in Patients With Hailey-Hailey Disease. JAMA Dermatol. 2018 Mar; 154(3): 362–363.
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