10.5826/dpc.1201a42
text/xml
2160-9381
1775
en
Prashant Durgapal
https://creativecommons.org/licenses/by-nc/4.0
Dermatology Practical & Conceptual
non-cicatricial
Graham-Little-Piccardi-Lassueur Syndrome With Concomitant Mucocutaneous Lichen Planus: Rare Presentation in a Man
Text.Serial.Journal
https://dpcj.org/index.php/dpc
Research Letter
2022-02-02
2021-05-05
2022-02-02
2022-02-09
e2022042
Dermatol Pract Concept
Prashant Durgapal
2022/02/02
10.5826/dpc.1201a42
e2022042
Department of Pathology, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India
2160-9381
non-cicatricial
en
e2022042
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. DOI: 10.4103/0974-7753.82129. PMID: 21769233. PMCID: PMC3129120.
Graham-Little-Piccardi-Lassueur Syndrome With Concomitant Mucocutaneous Lichen Planus: Rare Presentation in a Man
https://dpcj.org/index.php/dpc/article/download/1775/1496
    Graham-Little-Piccardi-Lassueur Syndrome With
    Concomitant Mucocutaneous Lichen Planus: Rare
                Presentation in a Man
              Avita Dhiman1, Priyanka Sangwan1, Neirita Hazarika1, Prashant Durgapal2


1 Department of Dermatology, Venereology and Leprosy, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India
2 Department of Pathology, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India



Key words: alopecia, cicatricial, lichen planopilaris, male, non-cicatricial
Citation: Dhiman A, Sangwan P, Hazarika N, Durgapal P. Graham-little-piccardi-lassueur syndrome with concomitant mucocutaneous
lichen planus: rare presentation in a man. Dermatol Pract Concept. 2022;12(1):e2022042. DOI: https://doi.org/10.5826/dpc.1201a42
Accepted: July 14, 2021; Published: January 2022
Copyright: ©2022 Dhiman et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License
BY-NC-4.0, https://creativecommons.org/licenses/by-nc/4.0/, which permits unrestricted noncommercial use, distribution, and reproduction
in any medium, provided the original authors and source are credited.
Funding: None.
Competing interests: None.
Authorship: All authors have contributed significantly to this publication
Corresponding author: Neirita Hazarika, MD, Additional Professor, Department of Dermatology, Venereology and Leprosy,
All India Institute of Medical Sciences, Rishikesh Uttarakhand, India. E-mail: neirita.derma@aiimsrishikesh.edu.in




Introduction                                                               pubic hair, along with multiple, pruritic, violaceous lesions
                                                                           over face, trunk for the last 6 years. He also complained
Graham-Little-Piccardi-Lassueur syndrome (GLPLS)                           of pain and burning sensation in his mouth for the past
described by Piccardi in 1913 is a rare variant of lichen                  6 months; associated history of recurrent, multiple, painful
planopilaris, characterized by a triad of multifocal cicatricial           swellings in bilateral axillae, groin in the last 2 years; 3 of
alopecia of the scalp, non-cicatricial alopecia of axillae, pubis          such episodes occurred in the previous 3 months. He also
and lichenoid follicular eruption [1]. The exact etiology is               reported history of 3 episodes of itchy, burning reddish lesion
unknown, but cell-mediated immunity may play a role. Over                  over abdomen, each time after taking painkillers.
50% of patients with GLPLS presents with at least 1 episode                    Cutaneous examination revealed scarring alopecia of
of cutaneous or mucosal lichen planus (LP) [1]. The progres-               whole scalp with few patches of uninvolved hair in occipital
sion of GLPLS is variable, and results in irreversible cicatricial         area (Figure 1A) and non-scarring hypotrichosis of axillary,
alopecia causing significant psychosocial distress [1]. Herein             beard area (Figure 1B,1C) and pubic region. Multiple poly-
we report a case of GLPLS in a male with mucocutaneous LP                  porous comedones and bridging scars were also seen in axillae,
and incidental findings of hidradenitis suppurativa (HS) and               (Figure 1B) and groin. Multiple violaceous, flat-topped papules
fixed drug eruption (FDE).                                                 over face (Figure 1C), trunk with lichenoid follicular papules
                                                                           over upper back (Figure 1D) were noted. Buccal mucosa showed
                                                                           whitish, reticular plaques (Figure 1E). Single, well-defined, oval
Case Presentation
                                                                           hyperpigmented macule was noted over abdomen (Figure 1F).
A male in his late forties presented with progressive loss of                  Investigations revealed positive serology for hepatitis
scalp hair over thirty years and history of loss of axillary and           C virus (HCV) with aspartate aminotransferase level of 131U/L,



Research Letter | Dermatol Pract Concept. 2022;12(1):e2022042                                                                              1
Figure 1. (A) Scarring alopecia of the scalp with few hair follicles at occipital hair line. (B) Non scarring alopecia of axilla with polyporous
comedones and scarred tracts. (C) Non scarring alopecia of beard area. (D) Multiple pinpoint hyperpigmented follicular papules over upper
back. (E) Reticulate whitish plaque over left buccal mucosa with surrounding hyperpigmentation. (F) Single well-defined oval hyperpigment-
ed macule over right hypochondrium region (arrow).




Figure 2. (A) Hisotopathological examination of a 3-mm skin punch biopsy from scalp showing perifollicular lymphocytic infiltrate (arrow)
(H&E, 100x). (B) Trichoscopy from occipital area showing perifollicular casts (star), homogenous white area suggestive of scarring (arrow),
and faint perifollicular erythema (triangle) (Dermlite DL3, polarized mode, ×10 magnification).



alanine aminotransferase 76 U/L, alkaline phosphatase                     and peri-pilar casts (Figure 2B). Based on clinical, dermoscopic
of 301 U/L; other lab values were within normal ranges.                   and histopathological features, a diagnosis of GLPLS with
Scalp biopsy showed perifollicular lymphocytic inflammation               quiescent HS, FDE and concurrent HCV infection was made.
(Figure 2A). Trichoscopy of occipital scalp revealed loss of hair             Oral drug provocation test to ascertain cause of FDE was
follicles with perifollicular scales, erythema, hyperpigmentation,        refused by the patient. Dermatological treatment was initiated



2                                                                             Research Letter | Dermatol Pract Concept. 2022;12(1):e2022042
with topical tacrolimus for scalp and facial lesions, topical    reported because of extreme rarity of presentation in a male
triamcinolone acetonide for oral lesions and clindamycin gel     with concomitant HCV infection and incidental findings of
for axillae and groin. Patient was counseled about the per-      HS and FDE.
manent hair loss in scalp and to use painkillers with caution.
                                                                 Informed consent: Written informed consent for publication
HCV infection was managed by gastroenterology depart-
                                                                 of clinical details and clinical images was obtained from the
ment as per protocol. Monthly follow-up showed moderate
                                                                 patient.
improvement in oral and cutaneous lesions of LP, no flare in
HS lesions and no further episodes of FDE.
                                                                 References
Conclusions                                                       1. Brar B, Khanna E, Mahajan BB. Graham Little Piccardi
                                                                     ­L asseur Syndrome: A Rare Case Report with Concomitant
GLPLS is a rare entity. Associations with Hepatitis B virus           ­Hypertrophic Lichen Planus. Int J Trichology. 2013;5:199–200.
                                                                       DOI: 10.4103/0974-7753.130403. PMID: 24778531. PMCID:
vaccination, androgen insensitivity, and HLADR-1 genetic
                                                                       PMC3999651.
susceptibility syndrome have been described [2]. ­Concomitant
                                                                  2. Pai VV, Kikkeri NN, Sori T, Dinesh U. Graham-little piccardi
HCV infection associated with classical LP was seen in our             lassueur syndrome: an unusual variant of follicular lichen planus.
case. GLPLS predominantly affects middle-aged women                    Int J Trichology. 2011;3:28–30. DOI: 10.4103/0974-7753.82129.
[1]; male cases of GLPLS are rare. This case of GLPLS is               PMID: 21769233. PMCID: PMC3129120.




Research Letter | Dermatol Pract Concept. 2022;12(1):e2022042                                                                         3
