Acrokeratoelastoidosis as an example of marginal papular acrokeratoderma with prominent elastorrhexis

Acrokeratoelastoidosis (AKE) is a rare condition described by Oswaldo Costa in 1953 [1]. Clinically, it is characterized by the presence of hyperkeratotic or umbilicated papules located on the margins of palms and/or soles. The first lesions usually develop in childhood or early adulthood and gradually increase in size and number, become grouped or form plaques [1–3]. The prominent histological feature of the condition is the diminished density and fragmentation (elastorrhexis) of elastic fibres, which can be detected using Verhoeff-van Gieson or orcein staining [1–3].
A 37-year-old Caucasian woman was admitted to the Department of Dermatology for evaluation of asymptomatic papular lesions located on the margins of hands and feet. The first papules had developed when the patient was 20 years old and had gradually increased in number. The lesions had been initially diagnosed as viral warts and had been treated with keratolytics and several sessions of cryosurgery with no improvement. Family history for similar skin changes was unremarkable. Several years prior to hospitalization, the patient had been diagnosed with ectopic gastric mucosa in the oesophagus, but was otherwise healthy. On physical examination, flesh-coloured papules of 2–4 mm in diameter were present along the margins of both hands accompanied by slight hyperkeratosis of the palms (Figures 1 A, B). Additionally, several hyperkeratotic papules were present at the line of transgredience between the dorsal and plantar surface of feet. Routine laboratory work-up was within normal limits. A 5-mm punch biopsy was performed from one of the papules, which revealed prominent irregular acanthosis, papillomatosis, hypergranulosis, focal hyperkeratosis and scarce perivascular inflammatory infiltration in the dermis with slight epidermotropism (Figure 2 A). Verhoeff-van Gieson staining revealed reduced density of elastic fibres with elastorrhexis (Figure 2 B). On the basis of clinical presentation and histological image the diagnosis of AKE was made. The patient was informed about the benign nature of the condition and she refused to try other treatment options.
AKE is considered to be a very rare disease, but its actual incidence may be underestimated. Over 50 cases have been reported so far. The number of articles has risen significantly in the last decade, with 18 cases reported in 2008–2018. Interestingly, recent reports include predominantly females (a female to male ratio of 7 : 2)...


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