PubMed · 9640547
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J C English, M L McCollough. 1998. Hereditary papulotranslucent acrokeratoderma.. https://pubmed.ncbi.nlm.nih.gov/9640547/
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A 45-year-old black woman presented with a chief complaint of an increasing number of "light spots" on her face, upper trunk, and legs. She had a 4-year history of a pruritic eruption on the dorsum of her hands. The eruption was particularly pruritic in the summer months. Other family members, including her sister and her daughters, reportedly had a similar dermatologic problem. The patient had been previously evaluated and biopsied by another dermatologist. The earlier biopsy was nondiagnostic, however, and she presented for further evaluation of this problem. On physical examination, the patient had hypopigmented macules along her jawline (Fig. 1), lateral neck, and upper chest. She had similar hypopigmented macules on her thighs. She had hyperkeratosis of the palmoplantar surface of her hands and feet. The dorsum of her hands had numerous coalescing, shiny, flat-topped, hypopigmented papules (Fig. 2), and several of her fingernails had distal, V-shaped notching. A punch biopsy from a papule on the dorsum of her hand was obtained. The epidermis had corps ronds present with focal areas of acantholysis above the basal layer (Fig. 3). The dermis had sparse, superficial, perivascular infiltrates composed of lymphocytes and histiocytes. These changes were consistent with our clinical diagnosis of Darier's disease (keratosis follicularis).
This article summarizes Louis H. Winer's description of the dilated pore and some of the pertinent literature about the dilated pore. To better understand the discussion that follows, there is a brief review of the histology of the follicular infundibulum and isthmus, then a discussion of the architectural and cytologic differentiation of proliferations that reputedly differentiate toward the infundibulum. These other proliferations with their original photomicrographs are discussed rather extensively to support the argument that they do not differentiate either architecturally or cellularly toward the infundibulum. I then present evidence that contradicts those authors who claim that Winer's dilated pore is only a cyst and conclude that it is a neoplasm sui generis and the only neoplasm of the skin that differentiates architecturally and cytologically towards the infundibulum (the infundibuloma).
The nail unit may reflect a dermatological disease by its own. Actually, its involvement may account for the existence of a skin disease that never showed before (i.e. lichen planus). But since the nail apparatus has a limited repertoire of clinical expressions (hyperkeratosis, onycholysis, paronychia...), the diagnosis may be sometimes difficult even for the most trained physician. Nail involvement may be however an additional clue in the diagnosis of an atypical skin disease. In that case, it might help in the final correct diagnosis provided that the physician did not forget that clinical examination of the nails is part of a dermatological examination.