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A Graham-Little-Piccardi-Lassueur syndrome accompanied by lichen planus pigmentosus

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Advances in Dermatology and Allergology 2, April/2021 340

This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International (CC BY-NC-SA 4.0).

License (http://creativecommons.org/licenses/by-nc-sa/4.0/)

Letter to the Editor

Address for correspondence: Fatma Erden MD, Department of Dermatology and Venereology, Atatürk Education Research Hospital, Yıldırım Beyazıt University, Üniversiteler Mahallesi Bilkent Caddesi No. 1, 06800 Çankaya, Ankara, Turkey, phone: +90 (0312) 291 25 25, +90 506 751 61 73, e-mail: doktor_ayyildiz@hotmail.com

Received: 13.03.2019, accepted: 15.07.2019.

A Graham-Little-Piccardi-Lassueur syndrome accompanied by lichen planus pigmentosus

Fatma Erden1, Aysel Çolak2, Hatice Karagoz3

1Department of Dermatology and Venereology, Atatürk Education Research Hospital, Yıldırım Beyazıt University, Ankara, Turkey

2Clinical Pathology Department, Ankara Numune Education and Research Hospital, Ankara, Turkey

3Internal Medicine Department, Acıbadem Kayseri Hospital, Kayseri, Turkey

Adv Dermatol Allergol 2021; XXXVIII (2): 340–342 DOI: https://doi.org/10.5114/ada.2021.106216

Graham-Little-Piccardi-Lassueur syndrome (GLPLS) is a rare variant of lichen planopilaris characterised by non- cicatricial hair loss of pubis and axilla, keratotic papules that are like keratosis pilaris follicles located on the trunk and extremities and cicatricial alopecia on the scalp [1].

However, it is not necessary for these three features to coincide. In this case, we aimed to present a 58-year-old patient with GLPLS accompanied by lichen planus pig- mentosus unlike the usual GLPLS.

A 58-year-old woman admitted to the hospital with a 2 years’ history of pruritus and spots which began on the scalp and axilla and then spread to the neck and chest. Shortly after these complaints, she reported alo- pecia involving her scalp, axillae and genitalia. She was postmenopausal for 5 years. She did not have any prob- lem in her past medical history and family history. She was not taking any medication. Dermatological examina- tion revealed locally brown and erythematous reticular patches on the axilla and both lateral necks and under the breasts. There were rare vellus type hair on the scalp, axilla and genital area without atrophy and nails were natural. Oral mucosa, genital mucosa and other system examinations were normal. Her laboratory findings in- cluding hemogram and biochemical values were also normal. The biopsies were taken from the lesions on the scalp and trunk with a pre-diagnosis of lichen planus. His- topathological examination revealed superficial perivas- cular, lichenoid interphase changes and melanophages in the lesion taken from the trunk and perifollicular lym- phocytic infiltration in the lesion taken from the scalp.

The patient was diagnosed as GLPLS according to the clinical and histopathological evaluation. After diagnosis, the patient received acitretin 25 mg/day and also topical

hydrocortisone butyrate 2 × 1 therapy for the trunk le- sions (Figures 1, 2).

At 3-month follow-up of the patient, we determined the regression of the lesions on the axilla, both lateral necks and under the breasts with a post inflammatory pigmentation.

The disease was originally defined in 1914 by Picca- rdi, who described a case of progressive cicatricial scalp alopecia, non-cicatricial alopecia in the axilla and pubic area, and follicular papules on the trunk and extremities [2]. Later, in 1915, Graham Little published a similar condi- tion defined as “folliculitis decalvans et atrophicans”. For this reason, the disease is called as GLPLS. The disease is accepted as a form of lichen planus due to histopatho- logical and immunofluorescence findings and clinical similarity [3, 4].

The condition is often observed in postmenopausal women and the ages of 30–70 years. It is see rarely in men [5]. Scarring alopecia of the scalp is often the first manifestation; but rarely, the chronological course of the clinical manifestations of GLPLS may vary. In our patient, scalp lesions were first manifestations similar to most cases, too [6].

In addition, our patient had lichen planus pigmento- sus, unlike other clinical presentations we were used to see in GLPLS.

Lichen planus pigmentosus is a rare variant of lichen planus characterized by hyperpigmented, brown macules in sun-exposed areas and flexural folds, which is often not associated with GLPLS [7, 8].

Although the aetiology of the syndrome is unknown;

autoimmunity, genetic factors, HBV vaccination, vitamin A deficiency, hormone dysfunction and androgen insensi- tivity syndrome are implicated [9–11]. Any pathology was

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Advances in Dermatology and Allergology 2, April/2021

A Graham-Little-Piccardi-Lassueur syndrome accompanied by lichen planus pigmentosus

341 detected in both systemic examination and laboratory

examinations of our patient.

In conclusion, GLPLS is a chronic and difficult disease to treat. Topical, systemic or intralesional steroids, reti- noids, hydroxychloroquine, phototherapy, cyclosporine, tacrolimus, tetracycline, griseofulvin, thalidomide, dap- sone and mycophenolate mofetil may be used in the treatment [12]. Chosen treatment and response varies from patient to patient. In conclusion, this case is re- ported because of a rare GLPLS case with lichen planus pigmentosus.

Conflict of interest

The authors declare no conflict of interest.

References

1. Yorulmaz A, Artuz F, Er O, et al. A case of Graham-Little- Piccardi-Lasseur syndrome. Dermatol Online J 2015; 16: 21.

2. Piccardi G. Cheratosi spinulosa del capillizio a suoi rapporti con al pseudo-pelade di Brocq. Giornale Italiano della Malat- tie Veneree e della Pelle 1914; 49: 416.

3. Graham-Little EG. Folliculitis decalvans et atrophicans.

Br J Dermatol 1915; 27: 183-5.

4. Zegarska B, Kallas D, Schwarts RA, et al. Graham-Little syn- drome. Acta Dermatovenerol Alp Panonica Adriat 2010; 19:

39-42.

5. Pai VV, Kikkeri NN, Sori T, et al. Graham-Little Piccardi Las- sueur syndrome: an unusual variant of follicular lichen pla- nus. Int J Trichology 2011; 3: 28-30.

6. Pittelkow MR, Daoud MS. Lichen planus. In: Fitzpatrick’s Der- matology in General Medicine. 7th ed. Wolff K, Goldsmith LA, Figure 1. There were rare vellus type hair on the scalp (A) and axilla (B) without atrophy. There were brown and locally erythematous reticular patches on the axilla (B) and both lateral necks (C) and under the breasts (D)

Figure 2. Histopathological examination revealed perifollicular lymphocytic infiltration in the lesion taken from the scalp (A) (H&E, 4×) and superficial perivascular, lichenoid interphase changes and melanophages in the lesion taken from the trunk (B) (H&E, 10×)

A

A

B

B

C

D

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Advances in Dermatology and Allergology 2, April/2021 342

Fatma Erden, Aysel Çolak, Hatice Karagoz

Katz SI, et al. (eds.). McGraw-Hill Professional, New York, NY 2007; 247.

7. Andrews’ Diseases of the Skin. 9th ed. Odom RB, James WD, Berger TG (eds). WB Saunders Company, Philadelphia 2000;

274-5.

8. Saray Y, Güleç AT, Seçkin D. Lichen planus pigmentosus:

report of four cases. Turkiye Klinikleri J Dermatol 2004; 14:

222-6.

9. Vega Gutiérrez J, Miranda-Romero A, Pérez Milán F, et al.

Graham Little-Piccardi-Lassueur syndrome associated with androgen insensitivity syndrome (testicular feminization).

J Eur Acad Dermatol Venereol 2004; 18: 463-6.

10. Bardazzi F, Landi C, Orlandi C, et al. Graham Little-Piccardi- Lasseur syndrome following HBV vaccination. Acta Derm Venereol 1999; 79: 93.

11. Karaca N, Ertam I, Türk BG, et al. Graham-Little Piccardi- Lassueur syndrome. Turkiye Klinikleri J Dermatol 2012; 22:

47-50.

12. Kang H, Alzolibani AA, Otberg N, et al. Lichen planopilaris.

Dermatol Ther 2008; 21: 249-56.

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