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      Graham–Little–Piccardi–Lasseur Syndrome

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      Indian Dermatology Online Journal
      Wolters Kluwer - Medknow

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          Abstract

          Sir, A 22-year-old male presented to us with the chief complaint of multiple pruritic follicular keratotic and spinous papules initially over wrists and forearms and neck, gradually involving the whole body, sparing face, palms, and soles since past 1 year. Over the past 3 years, the patient had noted patchy hair loss that developed and progressed slowly. On examination, sparse hair were present in axillary and pubic area and patches of scarring alopecia with shiny skin on scalp without erythema were apparent on the scalp [Figure 1]. Multiple violaceous follicular keratotic papules were present on the neck, lower back, and arms [Figure 2]. Axillary and pubic hair were sparse with a history of recurrent episodic shedding since the last 3 years [Figure 3]. The patient denied any constitutional symptom. A general physical examination and systemic examination found nothing relevant. No specific laboratory data were found. On histopathological examination (HPE) of the scalp, there was hyperkeratosis, mild acanthosis, and follicular plugging with dense perifollicular lichenoid inflammation, with vacuolar change of outer root sheath and occasional necrotic keratinocytes. Deep dermis showed perifollicular lamellar fibroplasia, collapsed fibrous sheaths, and reduction in hair follicular number [Figure 4]. Figure 1 Cicatricial (scarring) alopecia present on the scalp Figure 2 Follicular prominences were present on the neck Figure 3 Noncicatricial alopecia of the axilla Figure 4 Hematoxylin and eosin stain (×40) showed hyperkeratosis, mild acanthosis, and follicular plugging. Dense perifollicular lichenoid inflammation is present, with vacuolar change of outer root sheath and occasional necrotic keratinocytes. Interfollicular epidermis is spared. Deep dermis shows perifollicular lamellar fibroplasia, collapsed fibrous sheaths, and reduction in hair follicular number Based on the above characteristics, clinical and HPE features, a diagnosis of Graham–Little–Piccardi–Lasseur (GLPLS) syndrome was made. GLPLS is considered as a variant of lichen planopilaris. Its exact etiology is not known, but primarily involves an immune-mediated inflammatory reaction against the bulge region of hair follicles resulting in cicatricial alopecia.[1 2] The disease is chronic and slowly progressive and relatively rare, most reported patients with the syndrome are middle aged postmenopausal women.[3] Only a few case reports shows male patients, which may be because of less male patient reporting to hospital. The onset of the disease occurred most commonly in postmenopausal woman and an average of 26–52 year of age in all cases were seen. In two previous case reports, GLPLS was associated with other diseases such as androngen insensitivity syndrome and atopic dermatitis.[4] Histopathology reveals a dense perifollicular lymphocytic infiltrate thus, explaining scarring alopecia [Figure 4]. Lymphocytes extend into the basal layer and there is focal vacuolar alteration of the follicular basement membrane.[5] HPE close differentials are causes of scarring alopecia, viz. folliculitis spinulosa decalvans, discoid lupus erythematosus, and pseudopelade of brocq. 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.

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          Graham-Little Piccardi Lassueur Syndrome: An Unusual Variant of Follicular Lichen Planus

          Graham Little-Piccardi-Lassueur syndrome is a type of lichen planopilaris (follicular lichen planus) characterized by the triad of patchy cicatricial alopecia of the scalp, noncicatricial alopecia of the axilla and groin, and a follicular spinous papule on the body, scalp, or both. It is four times more common in females in the age group of 30-70 years. Only a few cases have been reported in literature wherein the disease has affected males. Herein we report a young male who presented with features of Graham Little-Piccardi-Lassueur syndrome.
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            Graham Little-Piccardi-Lassueur syndrome associated with androgen insensitivity syndrome (testicular feminization).

            Graham Little-Piccardi-Lassueur syndrome is characterized by the presence of cicatricial alopecia on the scalp, keratosis pilaris in the skin of trunk and extremities, and non-cicatricial hair loss in pubis and axillae. A frequent form of male pseudohermaphroditism is complete androgen insensitivity syndrome (CAIS), also known as testicular feminization syndrome. It refers to genetic males with XY karyotype who, owing to a lack of sensitivity in the peripheral androgenic receptors, develop a female phenotype. Axillary and pubic hair is typically scarce or absent. To our knowledge, this is the first case describing the association of the two processes. The presence of both processes in the same patient furthers our understanding of Graham Little-Piccardi-Lassueur syndrome as it rejects the influence of androgens in the alopecias accompanying this syndrome. The coincidence of non-cicatricial alopecia in axillary and pubic hair in both processes is also remarkable.
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              A case of Graham-Little-Piccardi-Lasseur syndrome

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                Author and article information

                Journal
                Indian Dermatol Online J
                Indian Dermatol Online J
                IDOJ
                Indian Dermatology Online Journal
                Wolters Kluwer - Medknow (India )
                2229-5178
                2249-5673
                Mar-Apr 2019
                : 10
                : 2
                : 180-181
                Affiliations
                [1] Department of Dermatology, Venereology and Leprosy, Post Graduate Institute of Medical Education and Research, Dr. Ram Manohar Lohia Hospital, New Delhi, India
                Author notes
                Address for correspondence: Dr. Gunjan Verma, Department of Dermatology, Dr. Ram Manohar Lohia Hospital, New Delhi 110001, India. E-mail: Gunjan.derma@ 123456gmail.com
                Article
                IDOJ-10-180
                10.4103/idoj.IDOJ_360_17
                6434740
                695842ac-6a99-420b-8326-a6356dca8000
                Copyright: © 2019 Indian Dermatology Online Journal

                This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.

                History
                : December 2017
                : June 2018
                Categories
                Letters to the Editor

                Dermatology
                Dermatology

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