Hereditary Papulotranslucent Acrokeratoderma in a 14-year-old Girl

Dear Sir, A 14-year-old girl presented with asymptomatic translucent papules on her palms and soles, which became prominent following water exposure; the lesions first appeared at age 12 and persisted without resolution. No associated pain, pruritus, or spontaneous regression was noted. There was no family history of similar lesions or history of atopy in the patient. On physical examination, small, flesh-colored papules were scattered on the palms [Figure 1], and they were accentuated after 10 min of water immersion [Figure 2]. The papules measured 1–3 mm in diameter and were distributed on the central palms. Dermoscopy after water immersion revealed white papules with overlying white-to-brown scales accentuated in the skin creases surrounded by edematous, whitish balloon-like areas [Figure 3]. A skin biopsy advised was refused by the patient. On the basis of clinical presentation and dermoscopy, a diagnosis of hereditary papulotranslucent acrokeratoderma was entertained. Given the asymptomatic nature of the condition, the patient was prescribed a keratolytic cream containing urea and salicylic acid for hyperkeratosis. While no significant improvement in lesion prominence was achieved, she reported no functional impairment or worsening of symptoms.Figure 1: Small, flesh-colored papules on the central area of the palm coalescing to form a plaqueFigure 2: Accentuation of the lesion after 10 min of water immersionFigure 3: Dermoscopy (DermLite DL4) after water immersion revealed white papules with overlying white-to-brown scales accentuated in the skin creases (arrows) surrounded by edematous, whitish balloon-like areas (square)Hereditary papulotranslucent acrokeratoderma (HPA) is a rare condition first described in 1973 as a variant of punctate palmoplantar keratoderma.[1] It typically manifests in adolescence and is characterized by persistent translucent papules accentuated by water exposure. It may involve the dorsum of hands progressing to palms with underlying trauma being implicated as causative. Histopathologically, it features hyperkeratosis, hypergranulosis, and acanthosis without inflammation, distinguishing it from inflammatory dermatoses.[1] HPA and aquagenic syringeal acrokeratoderma (ASA) share overlapping features, such as water-exacerbated lesions on the palms and soles, but they differ significantly in etiology, symptomatology, and histopathological findings.[2] HPA is a hereditary condition with an autosomal dominant pattern, typically manifesting during adolescence or early adulthood. In contrast, ASA is an acquired condition that can occur at any age, often presenting in younger individuals without a clear genetic link. Clinically, HPA lesions are persistent translucent papules that remain after water exposure, whereas ASA lesions are transient white plaques or papules that appear immediately after water immersion and resolve quickly upon drying. While HPA is asymptomatic, ASA is often accompanied by discomfort, including burning, itching, or edema, especially during water exposure. Histopathologically, HPA is characterized by hyperkeratosis, hypergranulosis, and acanthosis, with no abnormalities in sweat ducts. On the other hand, ASA shows dilated sweat ducts as a hallmark feature.[3] Dermoscopy can also be used to distinguish between the two conditions. In HPA, there are whitish balloon-like areas due to maceration of the thickened skin with white papules covered with the scale that involves skin creases. However, in ASA, there are large yellow well-defined globules due to the enlargement of sweat duct pores, and skin creases are spared.[4] There is no definitive treatment for HPA. Keratolytics, including topical urea, salicylic acid, tretinoin, or ammonium-based creams, may reduce hyperkeratosis but do not alter the underlying condition. Oral acitretin has also shown good results in anecdotal case reports.[5] Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.