Graham Little–Piccardi–Lassueur Syndrome with Frontal Fibrosing Alopecia in an Adolescent Girl: A Rare Presentation

Dear Editor,

An 18-year-old girl, born out of non-consanguineous marriage, from West Bengal, came with scarring alopecia of the scalp for 5 years, which was progressive in nature. She complained about severe itching of the scalp. She was of short stature and frail build. There was no significant past history or surgical illnesses. Family members had similar disease. There was no history of drug intake. Her menstrual history was regular. On cutaneous examination, she had scarring alopecia over the frontal [Figure 1a], temporal, and occipital areas of the scalp associated with scaling [Figure 1b] and non-scarring alopecia present over the axilla and pubic area [Figure 1c and d]. Follicular keratotic papules were present over the trunk, abdomen, upper limbs, and lower limbs [Figure 2a-d]. Eyebrows were affected (left > right) and eyelashes were normal. Breast development was normal. Her mucosa and nails were normal. Few furuncles were present over the forehead. Seborrheic dermatitis, graying of the scalp hair, and xerosis were also present. Breast development was normal. Mucosa and nails were normal. Dermoscopy (DermaLite DL4, 10×, contact/polarized) of the scalp showed a scattered white structureless areas with diffuse scaling, absence of follicular opening, blue-gray dots, and peripilar cast at the active margin [Figure 3a and b]. Dermoscopy of the eyebrows also showed diffuse scaling and peripilar casts [Figure 2d]. Dermoscopy of follicular papules of the trunk showed a few round-to-oval yellowish areas with keratotic follicular plugs surrounded by a violaceous hue [Figure 2c]. Her routine blood investigations were normal. Her hormonal profile and ultrasonography of the abdomen and pelvis were found to be normal. Biopsy of the scalp showed an unremarkable epidermis; the dermis showed diminished density of hair follicles and other adnexal structures and increased fibrosis [Figure 3a]. Hair follicles were predominantly in the anagen phase. There was mild-to-moderate perifollicular and periadnexal lymphocytic infiltrate [Figure 3b]. Clinico-dermoscopy findings and histopathology pointed toward lichen planopilaris (LPP)/frontal fibrosing alopecia (FFA) of the scalp. With the triad of LPP/FFA, noncicatricial alopecia over the axilla and pubis, and follicular keratotic papules over the trunk, we came to the diagnosis of Graham Little–Piccardi–Lassueur syndrome (GLPLS). The patient was counseled regarding the nature of the disease and was started on cyclosporine, but the patient was lost to follow-up. We are reporting this case due to the rarity of the disease, especially in the adolescent age group, as well as the presence of FFA.

Figure 1.

(a) Scarring alopecia of the scalp over the frontal area with bilateral eyebrow alopecia; (b) scarring alopecia over the frontal, temporal, and occipital areas of the scalp associated with scaling; (c) non-scarring alopecia present over the axilla; (d) non-scarring alopecia present over the pubic area along with follicular keratotic papules over the lower part of the abdomen; (e) follicular keratotic papules over the lower part of the trunk; and (f) over the lower limb

Figure 2.

Dermoscopy (DermaLite DL4, 10×, contact/polarized) of (a and b) scalp—scattered white structureless areas (red arrow) with diffuse scaling (black arrow), absence of follicular opening, blue gray dots (green arrow), and peripilar cast (blue arrow) at the active margin; (c) trunk—few round-to-oval yellowish areas with keratotic follicular plugs (blue star) surrounded by a violaceous hue (yellow arrow); (d) eyebrows—diffuse scaling (black arrow) and peripilar casts (blue arrow)

Figure 3.

Histopathology of (a) unremarkable epidermis; dermis shows the diminished density of hair follicles and other adnexal structures and increased fibrosis [H and E, 10x]; (b) mild-to-moderate perifollicular and periadnexal lymphocytic infiltrate [H and E, 40X]

GLPLS is an unusual variant of LPP characterized by the triad of cicatricial alopecia of the scalp, noncicatricial alopecia of the axilla and groin, and follicular spinous papules on the body, scalp, or both.[1] The average age of onset is 30–70 years and is four times more common in females.[2] The onset of symptoms can occur in any order with a predilection for the development of hyperkeratotic papules on the trunk and extremities before alopecia occurs in any location.[1] FFA is also increasingly recognized as a variant of LPP as far as histopathology and immunophenotyping are considered; the only difference being that FFA has symmetrical involvement of the frontotemporal and parietal regions[3] along with diffuse bilateral eyebrow alopecia[4] and the presence of lonely hairs. Our case had similar features suggesting FFA as a component of GLPLS. The exact etiology is unknown. The most common belief is that T-cell-mediated autoimmunity plays a major role although altered integrin expression also contributes to the cicatricial alopecia in GLPLS.[1] A few cases of familial pattern, association with hepatitis B, and androgen insensitivity syndrome have been reported.[1]

Dermoscopy of LPP shows blue-gray dots and perifollicular casts, while a dermoscopy of follicular papules shows multiple round-to-oval yellowish areas with keratotic follicular plugs surrounded by mild erythema in the report by Arisi et al.,[2] which is similar to our report.

Histopathology of early lesions of LPP shows perifollicular lymphocytic infiltrate at the level of the infundibulum and isthmus, along with vacuolar changes in the outer root sheath. The histopathology in our case showed cicatricial alopecia with a perifollicular lymphocytic infiltrate. More developed cases show perifollicular fibrosis with a characteristic hourglass configuration. In advanced cases, there is alopecia with vertically oriented elastic fibers that replace the destroyed hair follicle.[3] Different treatment modalities include topical and intralesional steroids, systemic corticosteroids, retinoids, cyclosporine, psoralen plus ultraviolet-A radiation (PUVA) therapy, and antimalarials.[3] In our case, we started her on cyclosporine, but she was lost to follow-up. To the best of our knowledge, only a single case has been reported in the pediatric age group.[5] Two cases of FFA as a component of this syndrome have also been reported in the literature.[4] However, the rarity of our patient being in the adolescent age group and having FFA in GLPLS prompted us to report this case.

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