Nekam Disease: A Rare Dermatosis and Challenging Treatment

A 39-year-old woman presented to the dermatology outpatient department with a coarse-looking face, and asymptomatic keratotic, violaceous papules, and plaques arranged in a linear pattern, symmetrically distributed on the trunk and extremities [Figure 1]. For the past seven years, she had been experiencing seborrheic eczema on the face, associated with conjunctivitis and blepharitis [Figure 2] without any altered laboratory tests. Occasionally, she complained of odynophagia and oral aphthous lesions. This clinical presentation was consistent with Nekam disease, also known as chronic lichenoid keratosis.[1-3]Figure 1: Erythemato-violaceous linear keratotic papular lesionsFigure 2: Seborrheic eczema on the face and oral aphthous lesionsThe erythemato-violaceous linear keratotic papules and plaques are characteristic of this condition.[1-3] Histopathology reveals a band-like lymphocytic infiltrate surrounding the perifollicular area, associated with keratinocyte necrosis and parakeratosis [Figure 3]. Treatment remains challenging due to the inadequate response to existing therapies, with oral retinoids being a potential treatment option.[1-3]Figure 3: (HE. 100×) Lichenoid infiltrate affecting the hair follicle, with destruction of the basement membrane, along with areas of epithelial flattening and follicular hyperkeratosisDeclaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that her name and initials will not be published and due efforts will be made to conceal her identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest. Use of artificial intelligence (AI) The preparation of this manuscript was carried out entirely by the authors without the use of artificial intelligence technologies.