Crusted Plaques in the Axillae

Localized Unilateral Hyperhidrosis and Neurofibromatosis Type 1: Case Report of a New Association

“…Cutaneous diseases described in association with localized hyperhidrosis include palmoplantar keratodermas, glomus tumor, blue rubber bleb nevus, sudoriferous nevus, POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein, skin changes) syndrome, speckled lentiginous nevus syndrome, Riley-Day syndrome, pachydermoperiostosis, Gopalan syndrome, causalgia, pretibial myxedema, Buerger disease and eccrine pilar angiomatous hamartoma, local injury and increased size of eccrine glands [5,23,24,25,26,27,28,29,30,31,32,33,34,35,36,37]. LUH, without other cutaneous findings, may occur as a symptom of disease of the central nervous system or peripheral neuropathy, such as stroke, trauma, syringomyelia, tabes dorsalis or pressure on a nerve root caused by a benign or malignant condition such as cervical rib, vertebral osteoma, mesothelioma or pulmonary carcinoma [3, 4,6,7,8,9, 11, 33, 38]. …”

“…Several cases of LUH have been reported in the literature without any apparent associated cause, and these cases were defined as idiopathic [10,11,12,13,14,15,16,17,18]. In these cases, hyperhidrosis is located mainly on the forearm and forehead of otherwise healthy people and is restricted to an area of less than 10 × 10 cm [38].…”

“…Since it was first described by Tarlov and Herz [1] in 1947, a limited number of cases has been reported in the medical literature [2,3,4,5,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25,26,27,28,29,30,31,32]. LUH has been attributed to the organic diseases of the nervous system such as cerebral infarction [2, 3], Holmes-Adie syndrome [4], Riley-Day syndrome [5] or has been reported to occur secondary to malignancies [6,7,8,9].…”

“…LUH has been attributed to the organic diseases of the nervous system such as cerebral infarction [2, 3], Holmes-Adie syndrome [4], Riley-Day syndrome [5] or has been reported to occur secondary to malignancies [6,7,8,9]. In some cases, however, an underlying pathology or an accompanying disorder cannot be determined, and these cases are considered as idiopathic [10,11,12,13,14,15,16,17,18]. Herein, we present a patient with LUH associated with neurofibromatosis type 1 (NF1).…”

Axillary Granular Parakeratosis

“…In the normal process of keratinization, profilaggrin, contained within keratohyalin granules, is broken down to filaggrin, which acts as a matrix for the aggregation of keratin filaments and is associated with the degradation of keratohyalin granules. 1,2,5,7,8 Metze and Rü tten 2 demonstrated by immunoelectron microscopy that the cytoplasm of granular parakeratotic cells was unreactive for filaggrin. This provides evidence that a defect in the processing of filaggrin probably occurs in this disorder.…”