Atypical Presentation of Kyrle’s Disease Showing Rapid Response with Surgical Debridement and Acitretin

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Kyrle’s disease (KD), also known as ‘hyperkeratosis follicularis et parafollicularis in cutem penetrans’ is a rare skin condition classified as a subtype of acquired perforating dermatosis.^[1] It is characterised by hyperkeratotic and ulcerated nodules and papules which may coalesce eventually to form circinate plaques and usually involves the extensor surface of limbs and trunk.^[2] The majority of case reports suggest an association with chronic systemic diseases (Stage 4 and 5 renal failure) and diabetes mellitus. Various treatment modalities are available with varying degrees of success.^[3] We report a case of KD where surgical debridement along with systemic Acitretin helped in early remission. This combination therapy has not been demonstrated in the literature according to our search.

A 50-year-old male presented to our outpatient department with a complaint of multiple itchy raised lesions over the neck, trunk, abdomen, and upper and lower limbs which had gradually increased in number over the last 6 months. The lesions were initially red and later became thicker and hyperpigmented with an increase in number and size. Few lesions had a purulent discharge. The patient denied any history of diabetes or renal disease. On examination, there were multiple well-demarcated, discrete, circumscribed, hyperkeratotic papules and plaques with a central hyperkeratotic plug distributed over the neck, upper back, bilateral upper and lower limb of size ranging from 0.5 × 0.5 cm to 3 × 3 cm [Figure 1a-d]. Lesions on the lower limb were larger, up to 8 × 6 cm, with grey-black crust which had underlying thick purulent discharge and granulation tissue at the base. Routine investigations including complete blood count, blood sugar levels, hepatic and renal function tests were normal. Human immunodeficiency virus, Hepatitis B and C serology were negative. Pus culture from leg lesion grew Staphylococcus aureus.

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Figure 1: Clinical picture. (a-d) At baseline, multiple well-demarcated, hyperkeratotic papules and plaques with a central hyperkeratotic plug distributed over the lower limb, upper back and upper limb.

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Histopathology from the plaque on the trunk revealed epidermis showing invagination containing basophilic degenerated material in a parakeratotic plug and underlying dermis with signs of chronic inflammation [Figure 2a and b]

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Figure 2: Histopathology. (a) Epidermis showing invagination containing basophilic degenerated material in parakeratotic plug (×10, haematoxylin and eosin [H&E]) and (b) underlying dermis showing chronic inflammatory infiltrate (×40, H&E).

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The patient was started on oral amoxicillin–clavulanic acid (625 mg) thrice daily along with sedating antihistamines. The papules and plaques were debrided surgically under local anaesthesia. Due to limited reduction in lesional thickness and the appearance of new lesions, he was started on Acitretin 25 mg once daily. After 6 weeks, noticeable improvement was seen with a reduction in size and number of lesions with residual atrophic scarring and hyperpigmentation [Figure 3 a-d]. The same dose of Acitretin was continued with no recurrence at 3^rd month of follow-up.

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Figure 3: Clinical pictures. (a-d) At 6 week follow-up, scarring and hperpigmentation present over the healed papules and plaques.

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KD is a rare dermatosis with characteristic clinical and histopathological features. It commonly presents as discrete or grouped, hyperkeratotic or ulcerated papules or nodules with a central keratin plug. It commonly affects females of age 30–50 years and involves the trunk, extensor surface of bilateral limbs and usually spares the face, mucous membranes, palms and soles.^[4] The exact aetiology is unknown; however, it is commonly associated with renal disease and uncontrolled diabetes. Uncommon associations include hepatic disease, congestive heart failure and hypothyroidism. However, no such association was present in our case. Various therapeutic modalities available include antibiotics, antihistamines, phototherapy, immunosuppressants, systemic/topical retinoids, keratolytics (urea and salicylic acid), cryotherapy and newer therapeutic modalities include drugs targeting interleukin receptors (IL-4/31) and Janus kinase pathways.^[3,5] However, all these modalities have varying success rates, and no single therapy is definitive. Acitretin alone typically shows improvement in 2–3 months, our patient improved within 6 weeks with the addition of surgical debridement by controlled removal of keratotic and infected material, promoting local resolution. Although the Koebner phenomenon is reported in KD, we did not observe lesion development at debrided sites.

To conclude, we suggest that surgical debridement may be combined with systemic therapy to achieve early remission.