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 Table of Contents  
CASE REPORT
Year : 2013  |  Volume : 5  |  Issue : 4  |  Page : 199-200  

Graham little piccardi lasseur syndrome: A rare case report with concomitant hypertrophic lichen planus


Department of Dermatology, Guru Gobind Singh Medical College and Hospital, Faridkot, Punjab, India

Date of Web Publication11-Apr-2014

Correspondence Address:
Era Khanna
Department of Dermatology, Guru Gobind Singh Medical College and Hospital, Faridkot - 151 203, Punjab
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0974-7753.130403

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   Abstract 

Graham Little Piccardi Lasseur Syndrome (GLPLS) is a rare variant of lichen planopilaris comprising of a triad of multifocal and patchy cicatricial alopecia of scalp, non-cicatricial alopecia of axillae and pubic region and keratotic follicular papules over a body. Its exact etiology is not known until date, but primarily involves an immune mediated inflammatory reaction against the bulge region of hair follicles resulting in cicatricial alopecia. We report an extensive and diffuse presentation of this rare syndrome in a middle-aged female with hypertrophic lichen planus (LP) present on malar region of face, etiologically relating GLPLS to LP.

Keywords: Cicatricial aloepecia, graham little piccardi lasseur syndrome, lichen planopilaris


How to cite this article:
Brar B K, Khanna E, Mahajan BB. Graham little piccardi lasseur syndrome: A rare case report with concomitant hypertrophic lichen planus. Int J Trichol 2013;5:199-200

How to cite this URL:
Brar B K, Khanna E, Mahajan BB. Graham little piccardi lasseur syndrome: A rare case report with concomitant hypertrophic lichen planus. Int J Trichol [serial online] 2013 [cited 2020 May 25];5:199-200. Available from: http://www.ijtrichology.com/text.asp?2013/5/4/199/130403


   Introduction Top


Graham Little Piccardi Lasseur Syndrome (GLPLS), a rare variant of lichen planopilaris, is a triad of cicatricial alopecia of scalp, non-cicatricial alopecia of axillae and pubic region and keratotic follicular spinous papules over a body. However, its coexistence with other variants of lichen planus (LP) has rarely been reported. The course of GLPLS is variable, ultimately culminating in permanent irreversible cicatricial alopecia and follicular lesions over a body, causing significant psychosocial distress.


   Case Report Top


A 35-year-old female patient presented with the chief complaint of multiple pruritic follicular keratotic and spinous papules initially over wrists and forearms, gradually involving whole body, sparing face, palms and soles since past 10 years, followed by appearance of hyperkeratotic violaceous itchy plaques over malar area of face with madrosis and scarring alopecia of scalp since 4 years. Multiple dilated follicular orifices plugged with keratotic debris were present on scalp with marked perifollicular violaceous hyperpigmention and complete loss of hair [Figure 1]. Axillary and pubic hair were sparse with history of recurrent episodic shedding since last 3 years. Violaceous pigmentation over buccal and gingival mucosae was seen along with melanonychia in fingernails. No history of photosensitivity or similar complaints in family.
Figure 1: Graham Little Piccardi Lasseur Syndrome. (a) Diffuse cicatricial alopecia of scalp with hyperkeratotoc follicular plugging. (b) Non-cicatricial alopecia of axilla with follicular prominences. (c) Multiple discreete keratotic follicular and spinous papules present all over abdomen. (d) Hyperkeratotic violaceous plaques of lichen planus on malar region of face and nasal bridge

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Routine investigations including viral markers for hepatitis B and C and serum antinuclear antibody levels were normal. Histopathological examination of hyperkeratotic alopecic plaque on scalp showed dilated follicular infundibula, with basket-weave hyperkeratosis in upper portion and compact hyperkeratotic plug in lower portion. Epithelium lining the follicular infundibulum showed marked hypergranulosis along with presence of vacuolar degeneration and apoptotic keratinocytes. Moderately dense perifollicular lymphocytic inflammation, lichenoid interface dermatitis and loss of sebaceous glands with follicular scarring were suggestive of lichen planopilaris with cicatricial alopecia. Biopsy from the follicular papules on body also revealed similar histopathological features consistent with lichen planopilaris [Figure 2]. Patient was put on oral retinoids in form of tablet isotane 20 mg once daily with regular follow up since past 2 months.
Figure 2: Histopathological examination, ×10. (a) Histopathology of hyperkeratotic alopecic plaque on scalp showing dilated follicular infundibula with compact hyperkeratotic plug in lower portion. Epitheliumlining the follicular infundibulum showed marked hypergranulosis with vacuolar degeneration of keratinocytes and lichenoid interface dermatitis with follicular scarring. (b) Histopathology of follicular keratotic papules on back showed dilated keratin plugged follicular opening with apoptotic keratinocytes and lichenoid interface dermatitis consistent with lichen planopilaris

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   Discussion Top


Graham Little Piccardi Lasseur Syndrome described by Piccardi in 1913, is a rare variant of lichen planopilaris. [1] In addition to the classical triad, a positive pull test for anagen hair can be present due to altered integrin expression in active lichen planopilaris lesions. [2],[3]

Graham Little Piccardi Lasseur Syndrome predominantly affects middle-aged females. [2] GLPLS is found to be associated with human leukocyte antigen-DR1 seen in familial cases of LP, thus etiologically relating GLPLS to LP. [4],[5],[6] It is estimated that over 50% patients with GLPLS present with at least one episode of cutaneous or mucosal LP, in the course of the disease. The pathogenetic mechanism of GLPLS involves the lymphohistiocytic infiltration of upper half of the pilosebaceous unit which is the main target of the disease, resulting in destruction of basal stem cells of the bulge region. [7]


   Conclusion Top


The extensive and diffuse presentation of GLPLS in this case in addition to concomitant hypertrophic LP affecting the face along with lesions of oral and nail LP depict a rare and severe presentation of LP and to our best knowledge, this is the first case of GLPLS to be reported from North India.

 
   References Top

1.Piccardi G. Keratosis spinulosa of the scalp in its relations with the pseudo-pelade of Brocq. ital g of skin and venereal diseases 1914;49:416.  Back to cited text no. 1
    
2.Odom RB, James WD, Berger TG, editors. Andrews' Diseases of the skin. 9 th ed. Philadelphia: WB Saunders Company. Lichen planus and related Conditions; 2000. p. 274-5.  Back to cited text no. 2
    
3.Assouly P, Reygagne P. Lichen planopilaris: Update on diagnosis and treatment. Semin Cutan Med Surg 2009;28:3-10.  Back to cited text no. 3
    
4.Srivastava M, Mikkilineni R, Konstadt J. Lassueur-Graham-Little-Piccardi syndrome. Dermatol Online J 2007;13:12.  Back to cited text no. 4
    
5.Tchernev G, Nenoff P. Antigen mimicry followed by epitope spreading: A pathogenetic trigger for the clinical morphology of lichen planus and its transition to Graham Lassueur Piccardi Little Syndrome and keratosis lichenoides chronica-Medical hypotheses or reality? An Bras Dermatol 2009;84:682-8.  Back to cited text no. 5
    
6.Daoud MS, Pittelkow MR. Lichen planus. In: Wolff K, Goldsmith LA, Katz SI, Gilchrest BA, Paller AS, Leffell DJ, editors. Fitzpatrick's Dermatology in General Medicine. 7 th ed. New York: McGraw Hill; 2008. p. 463-77.  Back to cited text no. 6
    
7.Matta M, Kibbi AG, Khattar J, Salman SM, Zaynoun ST. Lichen planopilaris: A clinicopathologic study. J Am Acad Dermatol 1990;22:594-8.  Back to cited text no. 7
    


    Figures

  [Figure 1], [Figure 2]



 

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