Coexistence of Darier's Disease and Acrokeratosis Verruciformis of Hopf

Abstract

Darier's disease and acrokeratosis verruciformis are inherited with the same pattern and they share similar clinical properties. We present a patient with both Darier's disease and acrokeratosis verruciformis. The patient had had brownish and skin-colored papules, initially on her face and neck, since the age of 25. Similar lesions had gradually spread to various parts of her body and lastly the dorsal aspect of her feet. On dermatological examination, brownish and skin-colored, 2 to 5 mm, keratotic papules were observed on her face, neck, both axillae, inframammary regions, and dorsa of the hands and feet. The nail examination revealed "V" shaped notches and longitudinal ridges on the fingers of both hands. The biopsy taken from the neck showed typical findings for Darier's disease. The biopsy from the dorsa of the left foot revealed the distinctive structures of acrokeratosis verruciformis. In this article the clinical and histopathological findings of these diseases are reviewed based on our patient.

Acrokeratosis verruciformis of Hopf in family

“…[ 3 ] Coexistence of AKV and DD has been reported in past. [ 4 ] In DD the site of predilection are the seborrheic area of the trunk and face where greasy, keratotic, foul-smelling, and skin-colored papules are seen with nail abnormalities in the form of fragility of the nail and red and white longitudinal stripes, which were absent in our cases. DD showed development of dyskeratosis of lesions in contrast to the lesions in AKV.…”

Congenital Wart-like Papules on Hands and Feet

“…Clinically, there are multiple, flat‐topped to convex, skin‐colored to brownish, nonconfluent papules, up to a few millimeters in diameter on the dorsa of the hands and feet bilaterally. Lesions may also involve the extensor surfaces of the arms and legs (3), but typically spare the forehead, scalp, flexures, and oral mucosa (5,6). Skin‐colored papules may be more easily palpated than seen (5).…”

“…A prominent granular layer and minimal dermal inflammatory infiltrate may also be observed (3). AKV is without parakeratosis, dyskeratosis, acantholysis, and vacuolization, which can aid in distinguishing it from other disorders of keratinization, such as Darier‐White (DW) disease (5,6).…”

“…Acral DW and AKV exhibit clinical similarities and debate exists over whether AKV is a distinct clinical entity or a mild form of DW (5). There have been reports of DW and AKV occurring within the same family and even within the same individual (5,6,9). The defective gene implicated in DW is ATP2A2 , a sarco(endo)plasmic reticulum Ca 2+ ATPase pump (9).…”

Acrokeratosis verruciformis of HOPF associated with dilated cardiomyopathy

“…[3] Coexistence of both these conditions has been documented in the past. [4] It is still not known why mutations in the widely expressed SERCA2 can manifest as a focal skin disease without any other systemic involvement. DCM is a disease which causes weakening of the heart muscle or myocardium resulting in a decrease in cardiac output.…”