Primary anetoderma in a young male involving palms, soles and the scalp: rarest of the rare

Abstract

Primary anetoderma is a rare idiopathic disease of the skin, characterized by circumscribed areas of loose skin, and loss of elastic fibers upon histopathologic examination. Two forms are traditionally distinguished, primary and secondary. Primary anetoderma occurs when there is no underlying associated skin disease, whereas the latter refers to an abnormal repair mechanism of preexisting skin lesions. We are reporting a case of primary anetoderma with lesions present all over the body, including the scalp, palms and soles, the sites that are not known to be involved in this condition.

Keywords: Elastolysis and elastorrhexis; palms; primary anetoderma; soles and scalp.

Figure 1

Bilaterally symmetrical multiple atrophic macules and plaques over the trunk and the upper limbs

Figure 2

Atrophic plaques with characteristic wrinkled surface

Figure 3

Lesions present over the penis and the scrotum

Figure 4

Lesions become flat on stretching

Figure 5

Skin colored dome-shaped papules on the ear lobe

Figure 6

Skin colored dome-shaped papules on the chin

Figure 7

Hyperpigmented macules and few crusted plaques on both the palms

Figure 8

Hyperpigmented macules on the sole

Figure 9

H and E-stained specimen showing mild dermal inflammatory infiltrate (x10)

Figure 10

H and E-stained specimen showing mild dermal edema with fragmented collagenized fibers (×40)

Figure 11

Verhoeff-Van Gieson stain showing reduced elastic fibres in the dermis (×10)

Figure 12

Verhoeff-Van Gieson stain showing reduced elastic fibres in the dermis (×40)