Acrokeratosis Verruciformis of Hopf Clinically Mimicking Epidermodysplasia Verruciformis.

Sir,

A 34-year-old female presented to skin outpatient department with multiple asymptomatic discrete hyperpigmented and hypopigmented lesions over scalp, face, neck, trunk, and both limbs since 2 years. No specific family history present. On examination, multiple hyperpigmented macules and papules were present over neck, back, face [Figure 1a], both upper and lower limbs and scalp [Figure 1b]. Multiple hypopigmented macules were present over back, chest, and limbs [Figure 2a and b]. Woods lamp examination of hypopigmented lesions did not revealed any fluroscence or accentuation. No orogenital involvement and nail changes noted. Biopsy was taken from hyperpigmented papule keeping epidermodysplasia verruciformis (EDV), Darier's disease and acrokeratosis verruciformis of Hopf (AKV) as differentials. Other biopsy was taken from hypopigented lesion with tinea versicolor and EDV as differential. Biopsy from both sites showed epidermis with hyperkeratosis, moderate acanthosis and papillomatosis. Rete ridges were elongated with circumscribed elevation of epidermis giving characteristic church spire appearance [Figure 3a and b] and mild perivascular lymphocytic infiltrates in upper dermis favoring AKV. Patient was advised topical retinoids over hyper pigmented lesions. Clinical improvement was noted in the form of reduced pigmentation after a month of follow-up.

Figure 1

Multiple hyperpigmented macules and papules over (a) neck, chest and face (b) scalp

Figure 2

Multiple hypopigmented macules over (a) chest (b) lower limbs

Figure 3

(a and b) Hyperkeratosis, moderate acanthosis and papillomatosis with elongated rete ridge and circumscribed elevation of epidermis giving characteristic church spire appearance (H and E, ×4 and ×10)

AKV is a rare genodermatosis, which develops at birth or during early childhood, but the onset may be delayed up to the fifth decade. It is more common in males as compared to females with a ratio of 5:1.3.[1] It results from a single dominant genetic defect with variable expressivity and is an allelic disorder arising from a mutation in ATP2A2.[2] Clinically, it presents as dry, rough, skin-colored or reddish-brown, flat-topped, or warty papules resembling flat warts on the dorsum of the hands and feet, on the knees, elbows, forearms, or lower legs. Forehead, scalp, flexures, and oral mucosa are never affected, as reported by Panja.[3] but scalp, forehead, neck, and upper trunk were mainly affected in our case. The palmar skin may be thickened and show punctate keratosis, pits, or punctiform breaks in dermatoglyphics. Nail involvement occurs in the form of longitudinal splitting, striations, and subungual hyperkeratosis.[4]

Classical AKV often occurs during childhood whereas the onset age of sporadic AKV is much later than that of classical AKV. Clinically typical morphology is seen at dorsum of hands and feet in classical variant while in sporadic it can affect other sites like face, scalp, trunk rather than classical sites as seen in our case. Positive family history with palmar pits and nail changes are seen in classical AKV but not in sporadic AKV.[5]

EDV is a rare autosomal recessive condition, clinically characterized by a combination of pityriasis Versicolor like hypopigmented lesions, verruca plana-like lesions, and seborrheic keratosis-like verrucous plaques.[6]

All the findings in our case were in favor of sporadic AKV with late age of onset, lesions over face, scalp, and trunk, without the involvement of palms, nails and insignificant family history. Such cases are reported but are uncommon.[7] Histopathologically, AKV shows exaggeration of the epidermal layer and thickening of the granular layer. These layers show a blunt, domed pattern within concavities of the wavy stratum corneum, so called “church spire.” There can be some degree of acantholysis, but not parakeratosis, dyskeratosis, or basal layer change.[3] Darier's disease, EDV, plane warts, and seborrheic keratoses, clinically simulate lesions of AKV, but they can be distinguished on the basis of histologic features. Hyperkeratosis and hypergranulosis with vacuolation and koiliocytes with mild to moderate dysplasia in the epidermis are consistent with the diagnosis of EDV while the absence of squamous and basaloid cells rules out the possibility of seborrheic keratosis. The only effective treatment of AKV is superficial ablation. Our case was unique with a sporadic variant of AKV in a female gender having onset age later in life, absent family history, no palmar pits or nail changes and involvement of neck, face, and scalp. Hypopigmented macules were also unique over trunk and extremity mimicking EDV.

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