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.

INTRODUCTION

Graham Little-Piccardi-Lassueur syndrome is a type of lichen planopilaris 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.[1] It is more common in females in the postmenopausal age group, with only a very few cases being reported in literature wherein the disease has affected males.[2] Here, we report a young male who presented with features of cicatricial alopecia of the scalp, noncicatricial alopecia of the pubic region and shoulder, and a follicular spinous papule over the body.

CASE REPORT

A 26-year-old male, from south India presented with history of loss of hair over the scalp, shoulder, and pubic region since 4 years. He also complained of small pigmented lesions over the abdomen, trunk, thighs, and forearm. History of itching, moderate in intensity over the pigmented lesions with mild scaling over the scalp was present. History of severe anxiety was regarding the nature of his skin condition. Treatment history included topical and systemic steroids, isotretinoin, without much relief.

On examination, scarring alopecia over the parietal area of the scalp around 8×8 cm, with few residual tufts of normal looking hair present, minimal scaling was noted [Figure 1]. Non-scarring alopecia was present over the pubic region and also over the shoulder [Figure 2]. Multiple follicular-oriented papules over the abdomen, trunk, thighs, and forearm were present [Figure 3].

Figure 1

Scarring alopecia over scalp

Figure 2

Non-scarring alopecia was present over the pubic region

Figure 3

Multiple follicular-oriented papules over the abdomen and trunk

A provisional diagnosis of lichen planopilaris was done. Routine investigation, including hemogram, blood sugar, renal and liver function tests, and thyroid function test were within normal limits. A biopsy from the scalp revealed features of scarring alopecia with dermis replaced by fibrous tissue with perifollicular and junctional lichenoid infilterate [Figure 4]. Based on the above findings, a diagnosis of Graham Little-Piccardi-Lassueur syndrome was made and the patient was started on treatment and counseled regarding the nature of his condition and advised regular follow up.

Figure 4

Dermis replaced by fibrous tissue with intense perifollicular and junctional lichenoid infilterate (H and E, ×40)

DISCUSSION

Piccardi in 1913, initially, described a case of progressive scalp cicatricial alopecia, noncicatricial alopecia in the axilla and pubic area, and follicular spinous papules on the trunk and extremities, to which he gave the name cheratosi spinulosa (keratotic spinulosa). Graham Little, later in 1915 published a similar case of a 55-year-old woman, referred by Lassueur of Lausanne, Switzerland, which was described as “folliculitis decalvans et atrophicans,”[3] followed by many similar reports later.

Graham Little–Piccardi-Lassueur syndrome is type of lichen planopilaris which presents in adults between 30 and 70 years and is four times more reported in females as compared to males.[1] LPP can be subdivided into three clinical variants: classical LPP, frontal fibrosing alopecia, and Graham Little–Piccardi-Lassueur syndrome.[2] In Graham Little-Piccardi-Lassueur syndrome, in addition to the triad, a positive pull test for anagen hair can be present.[1]

The exact etiology is unknown, but as it is considered a variant of lichen planus, the immunological mechanism, namely cell-mediated immunity may play a major role in triggering the clinical expression of disease.[4] Few isolated cases with a familial pattern, association with hepatitis B vaccination, and phenotypically female (genetically XY) patient with androgen insensitivity syndrome (testicular feminization) have been documented.[5-7] Altered integrin expression, which has been shown in active LPP lesions, could explain the phenomenon of anagen hair on pull test.[2]

Our case was young male who had in addition to scarring alopecia over the scalp, a non-scarring alopecia over the groin and shoulder. Non-scarring alopecia over the shoulder may be an associated finding, but not commonly reported.

Histopathologically, early lesions of lichen planopilaris show perifollicular lymphocytic infiltrate at the level of the infundibulum and the isthmus, along with vacuolar changes of the outer root sheath. More developed cases show perifollicular fibrosis and epithelial atrophy at the level of the infundibulum and the isthmus giving rise to a characteristic hourglass configuration. Advanced cases show alopecia with vertically oriented elastic fibers that replace the destroyed hair follicles. Similar findings were seen in our case. This end-stage scarring alopecia with no visible hair follicle is called pseudopelade of Brocq.[8]

Treatment is less effective for such types of lichen palnopilaris as compared to lichen planus, except in the early stage. Once scarring occurs, the hair growth is theoretically impossible.[2] Therefore, the treatment aims are to block the progression of the illness, prevent further alopecia, and symptomatic management. Various treatment modalities tried are topical, intralesional, and systemic corticosteroids, retinoids, cyclosporine, PUVA therapy, and antimalarials.[1] In our case the patient had good symptomatic improvement with pulsed systemic steroids, isotretinoin, and antianxiety drugs.

The course of the disease is often chronic and progressive with little potential for hair regrowth following follicular inflammation and destruction.[4] In conclusion, this case is reported because of its extreme rarity of presentation particularly in males.