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 Table of Contents  
CASE REPORTS
Year : 2022  |  Volume : 9  |  Issue : 1  |  Page : 27-30

Graham-Little-Piccardi-Lassueur syndrome—A rare case report with concomitant lichen planus pigmentosus and oral lichen planus in a pregnant female


Department of Dermatology, Smt. Kashibai Navale Medical College, Pune, Maharashtra, India

Date of Submission11-Aug-2021
Date of Decision14-May-2022
Date of Acceptance17-Jun-2022
Date of Web Publication26-Oct-2022

Correspondence Address:
Abhishek S Patokar
Department of Dermatology, Smt. Kashibai Navale Medical College, Pune, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijdpdd.ijdpdd_60_21

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  Abstract 

Graham-Little-Piccardi-Lassueur syndrome is a rare variant of lichen planopilaris. It is characterized by multifocal, patchy, cicatricial alopecia of the scalp, noncicatricial alopecia of axilla and pubic region, and keratotic follicular papules involving the trunk and extremities. Trichoscopy shows perifollicular erythema, tubular perifollicular scales, fibrotic white dots, and hair casts along the hair shafts. We are reporting this rare syndrome in a 26-year-old pregnant woman with concomitant oral lichen planus and lichen planus pigmentosus. This case is unique as no previous report of coexistence of these variants of lichen planus has been published.

Keywords: Cicatricial alopecia, keratotic follicular papules, lichen planus pigmentosus, oral lichen planus, trichoscopy


How to cite this article:
Patokar AS, Khandait GH, Chaudhari N, Khatu SS. Graham-Little-Piccardi-Lassueur syndrome—A rare case report with concomitant lichen planus pigmentosus and oral lichen planus in a pregnant female. Indian J Dermatopathol Diagn Dermatol 2022;9:27-30

How to cite this URL:
Patokar AS, Khandait GH, Chaudhari N, Khatu SS. Graham-Little-Piccardi-Lassueur syndrome—A rare case report with concomitant lichen planus pigmentosus and oral lichen planus in a pregnant female. Indian J Dermatopathol Diagn Dermatol [serial online] 2022 [cited 2023 Feb 8];9:27-30. Available from: https://www.ijdpdd.com/text.asp?2022/9/1/27/359692




  Introduction Top


Lichen planopilaris has three variants: Graham-Little-Piccardi-Lassueur syndrome (GLPLS), frontal fibrosing alopecia, and classic lichen planopilaris. However, fibrosing alopecia in a pattern distribution (FAPD) is a newly recognized variant of lichen planopilaris. FAPD shares the characteristic of both androgenetic alopecia and lichen planopilaris.[1] GLPLS typically affects the middle-aged women, particularly of the postmenopausal age group.[2] The course of GLPLS is variable, ultimately culminating in the permanent irreversible cicatricial alopecia and follicular lesions, causing a significant psychosocial distress.[3]


  Case Report Top


A 26-year-old married woman presented with multiple patches of hair loss over the scalp associated with itching since 5 years. It was associated with the thinning of hairs over axilla and groin since 4 years. She also noticed multiple, dark-colored, raised, itchy lesions over upper limbs, lower limbs, and trunk with few brown-colored flat lesions over the neck since 1 year. She also complained of the intolerance to spicy food since 6 months. She was 18-week primigravida woman. There was no history of photosensitivity and no family history of similar lesions.

Cutaneous examination revealed multiple patches of cicatricial alopecia predominantly over parietal and temporal regions of the scalp. Further examination revealed the noncicatricial alopecia over the axilla and pubis [Figure 1]. Hair pull test was positive. There were numerous, violaceous to hyperpigmented, perifollicular papules, over the extensor aspect of bilateral upper limbs, lower limbs, and abdomen. There were multiple, irregularly shaped, brown-colored macules over the neck, which adopted a linear configuration [Figure 1].
Figure 1: A, Noncicatricial alopecia over the axilla; B, multiple patches of cicatricial alopecia over the parietal and temporal regions of the scalp; C, oral lichen planus; D, numerous, violaceous to hyperpigmented, perifollicular papules, over the right arm; E, lichen planus pigmentosus

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Oral mucosal examination revealed a typical lace-like whitish reticular pattern on the violaceous plaque over the buccal mucosa [Figure 1]. Genital and nail examination was normal.

Trichoscopic examination of the scalp revealed absent follicular opening, peripilar casts, perifollicular grayish white scaling with perifollicular erythema. Trichoscopic examination of groin and axilla revealed the noncicatricial alopecia. Dermoscopy of lichen planus pigmentosus revealed a mixed pattern, combining diffuse brownish areas with dotted structures related to epidermal pigmentation and dermal melanophages, respectively. Dermoscopy of keratotic papules over the extremities revealed perifollicular casts, keratotic follicular plugging with mild perifollicular erythema, and the presence of vellus hairs that are coiled or looped [Figure 2].
Figure 2: Trichoscopy with Dermlite DL3N: A and B, absent follicular opening, peripilar casts, perifollicular grayish white scaling; C, mixed pattern, combining diffuse brownish areas with dotted structures; D, perifollicular casts, keratotic follicular plugging with mild perifollicular erythema, and the presence of vellus hairs that are coiled or looped

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Routine investigations, including hemogram, blood sugar, urine routine and microscopy, renal function test, liver function test, and thyroid function test, were within normal limits. Viral markers for hepatitis B were normal.

A skin-punch biopsy from the scalp revealed a marked reduction in the number of hair follicles and partial flattening of rete ridges. The papillary dermis shows fibroplasia with sparse superficial perivascular lymphocytic infiltrate. The interfollicular epidermis was spared. These findings were consistent with lichen planopilaris [Figure 3].
Figure 3: Histopathology (H&E, 40×): a skin-punch biopsy from the scalp revealed a marked reduction in the number of hair follicles, partial flattening of rete ridges, fibroplasia in papillary dermis, and sparse superficial perivascular lymphocytic infiltrate. The interfollicular epidermis was spared. These findings were consistent with lichen planopilaris

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Thus, the diagnosis of GLPLS associated with oral lichen planus and lichen planus pigmentosus was confirmed. The patient was counseled and reassured about the condition. We started her on topical tretinoin gel (0.04%) and topical mometasone furoate cream (0.1%) at the night along with narrow-band UVB (311 nm) light therapy twice a week. She is currently under the follow-up period.


  Discussion Top


GLPLS is a chronic, progressive, autoimmune disorder, first discovered by Piccardi in 1913 and 2 years later by Ernst Graham in 1915, referred by Lassueur of Lausanne, Switzerland.[4] It is four times more common in females of age group 30–70 years. Only a few cases of GLPLS are reported in males. One case was reported in a patient of androgen insensitivity syndrome.[5]

The exact etiology is unknown, but T cell-mediated immunity may play a major role in triggering the clinical expression of the disease. It usually occurs sporadically. However, a familial case of GLPLS syndrome has been described in a mother and daughter, both were positive for human leukocyte antigen-DR1.[6] It is associated with hyperthyroidism, hepatitis B virus vaccination, and autoimmunity. Altered integrin expression seen in the active lesions of lichen planopilaris explains the phenomenon of the positive anagen hair pull test.[7]

Trichoscopy may be useful in diagnosing, as well as monitoring the activity of all subtypes of lichen planopilaris. Moreover, together with a typical clinical presentation, it may substitute for a histological examination to avoid invasive procedures.[8] Differential diagnosis of GLPLS includes pseudopelade of Brocq, sarcoidosis, follicular mucinosis, folliculitis spinulosa decalvans, and keratosis pilaris atrophicans.[9]

Treatment is less effective for such types of lichen planopilaris as compared to lichen planus, except in the early stage. Treatment aims to block the progression of the disease, to prevent further alopecia, and for symptomatic management. Various treatment modalities tried are topical, intralesional, systemic corticosteroids, retinoids, cyclosporine, mycophenolate mofetil, antimalarials, and psoralen plus ultraviolet-A radiation therapy.[10] The coexistence of various variants of lichen planus is an alarming sign for a severe form of disease. So, a more aggressive and prompt approach is essential for its treatment. In our case, more conservative approach was followed because the patient was pregnant and wanted to continue her pregnancy.

This case seems to be interesting as no previous report of coexistence of three variants of lichen planus in a pregnant woman has been published.

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.



 
  References Top

1.
Ramanauskaite A, Trüeb RM Facial papules in fibrosing alopecia in a pattern distribution (cicatricial pattern hair loss). Int J Trichology 2015;7:119-22.  Back to cited text no. 1
    
2.
Verma G Graham-Little-Piccardi-Lasseur syndrome. Indian Dermatol Online J 2019;10:180-1.  Back to cited text no. 2
    
3.
Brar BK, Khanna E, Mahajan BB Graham Little Piccardi Lassueur syndrome: A rare case report with concomitant hypertrophic lichen planus. Int J Trichol 2013;5:199-200.  Back to cited text no. 3
    
4.
László FG Graham-Little-Piccardi-Lasseur syndrome: Case report and review of the syndrome in men. Int J Dermatol 2014;53:1019-22.  Back to cited text no. 4
    
5.
Vega Gutiérrez J, Miranda-Romero A, Pérez Milán F, Martínez García G Graham Little-Piccardi-Lassueur syndrome associated with androgen insensitivity syndrome (testicular feminization). J Eur Acad Dermatol Venereol 2004;18:463-6.  Back to cited text no. 5
    
6.
Christopher Hixon DO, Blattner CM, Daniel Hurd DO Graham-Little-Piccardi-Lassueur syndrome: A case report. J Am Osteopath Coll Dermatol 2015;33:36-7.  Back to cited text no. 6
    
7.
Pai VV, Kikkeri NN, Sori T, Dinesh U Graham-Little-Piccardi-Lassueur syndrome: An unusual variant of follicular lichen planus. Int J Trichology 2011;3:28-30.  Back to cited text no. 7
    
8.
Waśkiel A, Rakowska A, Sikora M, Olszewska M, Rudnicka L Trichoscopy in lichen planopilaris: An update. Dermatol Rev/Przegląd Dermatologiczny 2018;105:63-75.  Back to cited text no. 8
    
9.
Bissacotti SR, Elise TR, Martins PG, Melo MF, Maffei GI, Siegmann DE Graham-Little Piccardi Lassueur syndrome: Case report. An Bras Dermatol 2012;87:775-7.  Back to cited text no. 9
    
10.
Saha A, Seth J, Das A, Dhar S Graham-Little-Piccardi syndrome: A lens through beyond what is known. Int J Trichol 2016;8:173-5.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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