Sir,
An 11-year-old female child born of third-degree consanguineous marriage was brought with a history of crusted skin lesions on the scalp, face, and trunk for 6 months.
Examination revealed multiple hyperpigmented crusted annular papules and plaques with verrucuous surface, well-defined borders, and some satellite papules over the scalp, face, neck, chest, and abdomen. [Figure 1]a,[Figure 1]b,[Figure 1]c
Figure 1: (a) and (b) Multiple hyperpigmented, crusted papules and plaques with verrucuous surface over the face and neck. (c) Annular crusted plaques over the chest and abdomen showing satellite papulesThere were whitish soft plaques covering the tongue. The nose, ears, other mucosal surfaces, and nails were normal. Systemic exam showed no abnormality [Figure 2]a and [Figure 2]b.
Figure 2: (a) Whitish soft plaques over the tongue. (b) and (c) Normal nailsInitially, the differential diagnoses of lupus vulgaris and dermatophytosis were considered.
Scrapings from the plaques showed pseudohyphae of Candida. [Figure 3]
Figure 3: KOH mount (10%) of the (a) skin scrapings and (b) tongue swab, in 40× magnification showed pseudohyphae suggestive of Candidal originHence, with mucocutaneous candidiasis in mind, a panel of tests were conducted. No significant abnormality was noted in complete blood counts, thyroid profile, serum calcium, cortisol levels, ELISA for HIV, Mantoux test, chest radiography, and ultrasound scan of abdomen.
Biopsy from the crusted plaques showed hyperkeratotic and hyperplastic squamous epithelium with upper dermal infiltrate and fungal hyphae in the stratum corneum. [Figure 4]a,[Figure 4]b,[Figure 4]c. Candida albicans was cultured from the skin scrapings. [Figure 5] Thus a diagnosis of chronic mucocutaneous candidiasis (CMC) was made.
Figure 4: (a) Biopsy from crusted plaque stained with Haemotoxylin and Eosin under 10 × magnification showed hyperkeratotic and hyperplastic squamous epithelium with upper dermal infiltrate. (b) and (c) Fungal hyphae were seen in the stratum corneum under 40× magnificationFigure 5: Colonies of Candida albicans grown from the skin scrapings of the patientTreatment with 75 mg of daily oral fluconazole showed improvement in 2 weeks and complete clearance in 8 weeks. [Figure 6]a,[Figure 6]b,[Figure 6]c,[Figure 6]d,[Figure 6]e,[Figure 6]f,[Figure 6]g,[Figure 6]h,[Figure 6]i,[Figure 6]j,[Figure 6]k
Figure 6: Patient's response to daily oral fluconazole treatment. (a), (b) and (c) Response after 2 weeks of therapy. (d), (e) and (f) Response after 3 weeks of therapy. (g), (h), (i), (j) and (k) Response after 8 weeks of therapyRecurrences occurred 8 and 14 months later. They responded to an alternate day 2-week regimen of 75 mg of oral fluconazole. [Figure 7]a,[Figure 7]b,[Figure 7]c There was normal physical development with no recurrences up to seven years.
Figure 7: (a) and (b) Recurrence of cutaneous plaques after 8 months. (c) Recurrence of scaly plaque 14 months after initial presentationCMC is a group of disorders with persistent candidal infections of the skin, nails, and mucosa.[1] Scaly reddish annular cutaneous lesions resembling dermatophytosis or hypertrophic variety with verrucuous crusted plaques are seen. Florid paronychia with thickened brittle nails and a hypertrophic form of oral thrush may be present.
CMC is classified into five types, namely: idiopathic, autosomal recessive type with mucocutaneous lesions that improve with age, severe autosomal dominant type, late-onset adult type with thymoma, and endocrinopathy-associated type.
Our patient could fit in with either the autosomal recessive type or the one associated with endocrinopathy. The latter is known as Autoimmune Polyglandular Syndrome-1 (APS-1) or Autoimmune-Poly Endocrinopathy-Candidiasis-Ectodermal Dystrophy Syndrome (APECED) or Whitaker's syndrome.[2] Here, childhood onset of CMC is followed by autoimmune endocrine dysfunction involving the adrenals and parathyoids.[3] Hypogonadotrophic hypogonadism, hypopituitarism, diabetes mellitus; chronic active hepatitis, juvenile cirrhosis, pulmonary fibrosis, pernicious anemia, alopecia areata, vitiligo, keratoconjunctivitis, and dental enamel hypoplasia are other associations.
Systemic antifungals like ketoconazole, fluconazole, itraconazole, posaconazole and terbinafine are used in symptomatic treatment.[4],[5] Immunomodulators like levamisole, cimetidine, and zinc have also been tried. Follow-up screening for endocrinopathy and other diseases is essential.
This case was presented to highlight the characteristic clinical presentation in this patient along with the response to oral fluconazole. The recurrences highlight the importance of follow up especially in early detection of any subsequent systemic involvement.
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