Circumscribed acral hypokeratosis

Premalignant Circumscribed Palmar Hypokeratosis: A New Form of Circumscribed Palmar Hypokeratosis? Case Report and Literature Review

“…CPH is a dermatosis of unknown origin, first reported in 2002 [1], of which 47 cases (including the present one) have so far been reported [2,3,4,5,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25,26]. In our dermatopathology practice we have diagnosed CPH in 3 additional patients during the 7 last years [unpubl.…”

“…The clinical appearance of the lesions is stereotypical: they consist in a sharply circumscribed plaque with a erythematous, somewhat depressed surface, rimmed by a slightly hyperkeratotic border. The lesion is usually single, although multiple lesions (up to 14) [18] have occasionally been reported, affecting the palms and soles. The lesion is predominantly localized over the thenar or hypothenar eminences of the palm of the predominant hand; less commonly, it is seen on the fingers and the soles.…”

“…The underlying dermis is unremarkable. Some variations or additional features to this aspect have been reported, including a thin layer of parakeratosis [8,10,11], hypergranulosis [11,18], epidermal hyperplasia [11,12,18], a mild dermal inflammatory infiltrate [11], spongiosis [18] and dermal elastosis [1]. …”

“…CPH has been regarded as a benign clonal epidermal malformation [1]; however, this view has been debated because of the late onset of the disease [2]. Because CPH usually affects the dominant hand, it has been suggested that CPH could be an abortive form of epidermolysis bullosa, with the lesions resulting from local trauma [11]; however, only 6 of the previously reported patients gave a history of trauma [4,11,17,18], and a correlation with handedness is not invariable. Böer and Falk [12] suggested HPV infection of keratinocytes as being a possible (co)factor; however, only 2 cases documented the presence of HPV DNA (types 4 and 6) [12,18], whereas at least 10 additional cases (including our own) proved to be HPV-negative.…”

“…However, treatment is disappointing. Local steroids, retinoids and keratolytics are usually ineffective [3,8,10,17,18]. Remissions have occasionally been achieved with local applications of calcipotriene [1,9], cryotherapy [17] and photodynamic therapy [20].…”