Acrokeratoelastoidosis

Abstract

Acrokeratoelastoidosis is a rare palmoplantar keratoderma. We report the case of a 32-year old man with multiple keratotic papules and plaques on the peripheral margins of his hands and feet. Histopathologic evaluation showed compact orthokeratosis; Verhoeff van Gieson stain showed thin and fragmented elastic fibers. The differential diagnosis includes focal acral hyperkeratosis, which has a similar presentation but with normal elastic fibers. Treatment of acrokeratoelastoidosis is often disappointing, with minimal response to topical glucocorticoids, keratolytics, retinoids, cryotherapy, and lasers. Case synopsisHistory: A 32-year-old man presented to the Bellevue Hospital Center Dermatology Clinic for evaluation of multiple papules on his hands and feet that appeared over the past year. The affected areas were associated with a mildly pruritic and intermittently burning sensation. The lesions on his hands and feet developed around the same time and were increasing in number. The patient worked part-time as a construction worker and dishwasher and believed that the initial skin eruption occurred after exposures to various occupational chemicals and detergents. There was no history of trauma, hyperhidrosis, or prolonged exposure to the sun. Neither the patient nor his close contacts had a history of similar lesions. The patient was otherwise well with no relevant family or personal medical history. He was treated with urea 40% cream twice daily and clobetasol 0.05% ointment twice daily for three weeks without improvement. Physical examination:Multiple, 2-to-3-mm, tan, firm papules were clustered along the peripheral margins of the palms and soles. Laboratory: None.Histopathology: There is hyperkeratotic compact orthokeratosis. Within the dermis, a Verhoeff-van Gieson stain shows thinning and fragmentation of elastic fibers.

A sporadic case of knuckle pad‐like lesions

“…Neither curative treatment nor a tendency toward spontaneous resolution have been reported. Modest to poor outcomes have been achieved with local or systemic treatments, such as ablative lasers, glucocorticoids, salicylic acid or methotrexate, dapsone and retinoids [5]. Even after responding to therapy, lesions routinely relapse upon discontinuation of treatment [10].…”

“…As in the present case, when onset is mild, the patient might only become aware of the progression of the disease upon appearance of knuckle pad-like keratoderma at the dorsa of the finger joints. In general, such skin manifestations may be exacerbated by nonspecific triggers such as pregnancy, water contact, and acral hyperhidrosis, which may also make associated symptoms such as pruritus more prominent [4,5].…”

Ein sporadischer Fall knöchelpolsterartiger Keratodermien

“…Bislang wurde weder eine kurative Behandlung noch eine Tendenz zur Spontanremission berichtet. Mäßige bis schlechte Behandlungserfolge wurden mit lokalen oder systemischen Therapien wie ablativen Lasern, Glukokortikoiden, Salicylsäure oder Methotrexat, Dapson und Retinoiden erzielt [5]. Selbst nach Befundansprechen, treten nach Absetzen der Therapien routinemäßig Rückfälle auf [10].…”

“…Wie im vorliegenden Fall kann bei dezentem Initialbefund das Fortschreiten der Erkrankung erst bei Auftreten einer knöchelpolsterartigen Keratodermie an der Rückseite der Fingergelenke bemerkt werden. Grundsätzlich können solche Hautmanifestationen durch unspezifische Trigger wie Schwangerschaft, Wasserkontakt oder akrale Hyperhidrose verstärkt werden und assoziierte Symptome wie Juckreiz stärker hervortreten lassen [4,5].…”