Lichen planopilaris.

P, a 20-year-old laborer displayed initial symptoms of the disease in question when he was 10 years old. Initially he had an asymptomatic progressive loss of hair on the scalp. A couple of years later he had mild to moderate pruritis, and the appearance of slate-blue eruptions on the scalp and elsewhere on the body. This resulted in a complete loss of hair on the vault of the scalp, which led him to seek specialist opinion. Skin surface examination revealed the presence of grayish-blue acuminate follicular papules, disposed singly and in groups (plaques). The pilo-sebaceous orifices were conspicuously obliterated and filled by keratin plugs. Perifollicular erythema was a predominant feature on the scalp. The lesions were present over the scalp, around the neck, chest, back, axillae, groin and legs. Shiny atrophied scalp skin depicting scarring alopecia mimicking male-type baldness was a salient feature. In addition, it was studded with conspicuous acuminate papules in its center (Fig. 1a). The known nonhairy (glabrous) skin had classic lichen planus lesions (Fig. 1b). Hemotoxylin-eosin stained microsections prepared from typical lichen planus (LP) lesions over the abdomen and those of lichen planopilaris (LPP) of the scalp were simultaneously studied. The former revealed changes in the epidermis comprising of hyperkeratosis, increase in thickness of stratum granulosum, hydropic degeneration of the basal cell layer and band-like lympho-histiocytic infiltrate pressing against and invading the epidermis, while the latter revealed uniform atrophy of the epidermis and vacuolization of basal cells. The hair follicles were dilated and were filled with keratin plugs. In addition to fibrosis of the dermis, pigment laden microphages and lympho-histiocytic infiltrate was prominent. The follicles and the sebaceous glands were absent. However, arrectores pilorum and sweat glands were preserved (Fig. 2a,b).     

Lichen planopilaris presenting as truncal alopecia: a case presentation and review of the literature

Lichen planopilaris is an inflammatory condition of unknown etiology, characterized by violaceous folliculocentric papules and hair loss. Some clinicians consider lichen planopilaris to be a variant of lichen planus, but others believe it to be a separate disease entity. Many treatment modalities have been utilized, with varying degrees of success. We describe the case of a 63-year-old man who presented with widespread alopecia of the trunk and extremities and was subsequently diagnosed with lichen planopilaris.     

Lichen planopilaris: a clinicopathologic study

Three clinicopathologic variants of lichen planopilaris are described. The first is characterized clinically by individual keratotic follicular papules and histologically by a lichenoid inflammatory cell infiltrate confined to the follicular epithelium. The second variant consists of erythematous to violaceous plaques, some of which show follicular prominence; the histologic appearance is that of a lichenoid inflammatory cell infiltrate that affects both follicular and interfollicular areas. The third variant manifests as follicular papules of the scalp with concomitant or subsequent cicatricial alopecia. In this variant the histologic hallmark is a lichenoid, follicular and interfollicular inflammation, associated with or followed by scarring. Overlap among the three variants exists, and hence the concept of a disease spectrum ranging from pure follicular involvement without evidence of clinical scarring to cicatricial alopecia of the scalp is advocated.     

Myopericytoma as an unusual cause of scarring alopecia in a child

Myopericytoma is a benign tumor composed of myoid-appearing oval to spindle-shaped cells arranged in a concentric perivascular pattern of growth. It arises most commonly in the dermis or subcutaneous tissue of the extremities in adults, and presents as a well-circumscribed, slow-growing, painless firm tumor. We describe a case of myopericytoma with unusual clinical presentation as scarring alopecia in a 14-year-old girl without any underlying disease, who presented with a 2-month history of a pink patch with hair loss on her frontal scalp. Under the diagnosis of discoid lupus erythematosus, an incisional biopsy was also performed. The biopsy specimen revealed a myopericytoma characterized by a subcutaneous large vein-like structure surrounded by spindled, stellate, and cuboidal cells with multiple small branching vascular spaces. The overlying dermis was fibrotic with dilated vessels and the number of hair follicles was decreased. No features of lupus erythematosus were noted and a lupus band test was negative. Total excision was performed. No recurrence occurred in the following 2 years.     

Lichen planopilaris and frontal fibrosing alopecia: review and update of diagnostic and therapeutic features

Lichen planopilaris and frontal fibrosing alopecia are primary scarring alopecias where diagnosis can be suggested by clinical and trichoscopy features, especially in the early stages, but scalp biopsy is the standard exam for definitive diagnosis. Frontal fibrosing alopecia is considered a variant of lichen planopilaris, as the histopathological findings are similar, with a perifollicular lymphohistiocytic infiltrate, sometimes with a lichenoid pattern. A thorough clinical examination, trichoscopy and photographic documentation are essential to assess the evolution and therapeutic response. To date, there are no validated treatments or guidelines for these diseases, but there are recommendations that vary with the individual characteristics of each patient. This article presents a comprehensive review of the literature, including an update on topics related to the diagnosis, follow-up, histopathological aspects and available treatments for lichen planopilaris and frontal fibrosing alopecia, highlighting their similarities, differences and peculiarities.     

Follicular streamers (stelae) in scarring and non-scarring alopecia

Background: Follicular streamers are residual fibrovascular tracts representing the impermanent lower third of the hair follicle below the bulge region. Streamers are generally not counted in transverse alopecia samples as they may represent catagen/telogen (CT) follicles and vellus‐like (VL) follicles, or be mistaken as follicular scars. Design: We evaluated 22 non‐scarring alopecia cases, including alopecia areata (AA) and androgenetic alopecia (AGA), and 22 scarring alopecia cases, including follicular degeneration syndrome (FDS)/central centrifugal cicatricial alopecia and other scarring alopecia (OSA) disorders. We counted terminal follicular streamers found at a deep dermal level (L2) and followed them into a mid‐dermal level at the central follicular unit (FU) to determine their precise derivation. Results: We found streamers in 8/9 AA, 11/13 AGA, 6/12 FDS and 3/10 OSA cases. We counted a total of 74 streamers at L2, including 61 in non‐scarring alopecia cases (p < 0.001). At the more superficial FU level, 72% of streamers corresponded to CT follicles, 25% to VL follicles and 3% to follicular scars. Conclusions: Follicular streamers are found predominantly in non‐scarring alopecia cases. Streamers found at deep dermal or subcutaneous levels should be followed and identified at the FU level in order to obtain accurate follicular counts and follicular ratios needed for non‐scarring alopecia diagnosis.     

Lichen planopilaris unter dem Bild einer postmenopausalen frontalen fibrosierenden Alopezie (Kossard)

Zusammenfassung Es wird über eine 68j?hrige Patientin mit frontaler fibrosierender Alopezie und Lichen-planus-typischen Wangenschleimhautver?nderungen berichtet. Das kürzlich von Kossard beschriebene Krankheitsbild der postmenopausalen frontalen fibrosierenden Alopezie (PFFA) zeichnet sich durch einen progressiven Rückgang des frontotemporalen und parietalen Haaransatzes unter Ausbildung einer symmetrischen, bandf?rmigen narbigen Alopezie aus. Typischerweise erkranken Frauen in der Postmenopause. Die histopathologischen Ver?nderungen (perifollikul?res lymphozyt?res Entzündungsinfiltrat und Fibrose im Bereich des Isthmus und des Infundibulums, vermehrte Apoptose der Follikelkeratinozyten) sind identisch mit denen beim Lichen planopilaris. Die lokalisations- und alterstypischen Merkmale der PFFA sind nicht hinreichend, um das Krankheitsbild als eigenst?ndige Entit?t vom Lichen planopilaris eindeutig abzugrenzen. Unsere Beobachtung Lichen-planus-typischer Mundschleimhautver?nderungen bei einer postmenopausalen Patientin mit frontal fibrosierender Alopezie weist auf die M?glichkeit hin, da? es sich bei der PFFA um eine Variante des Lichen planopilaris mit frontaler Lokalisation handeln k?nnte. Andere Varianten des Lichen planopilaris sind die multifokale Form, die disseminierte Form (Lassueur-Graham-Little-Syndrom), die lichenoide Pseudopelade und jede Kombination dieser verschiedenen Manifestationsformen (”gemischte Form”). Wirksame Behandlungsmethoden der PFFA sind nicht bekannt. Ebenso problematisch ist die therapeutische Beeinflussung von Lichen-planopilaris-Formen, bei denen die Fibrose gegenüber der Entzündung im Vordergrund steht, doch scheint in diesen F?llen der Krankheitsverlauf selbstlimitierend zu sein. Eingegangen am 10. Dezember 1996 Angenommen am 11. April 1997     

Tissue expansion in cicatricial alopecia

A surgical approach for the treatment of cicatricial alopecia may be considered when the causal component has resolved and hair replacement is desired. Traditional procedures have been limited to scalp reduction and hair transplantation. The advent of expandable silicone implants under adjacent normal hair-bearing skin for the creation of scalp flaps offers another approach for scalp reconstruction. Tissue-expansion augmented scalp reduction of cicatricial alopecia is a surgical procedure for some patients who might not otherwise be candidates for surgical hair replacement.     

Possible role of the bulge region in the pathogenesis of inflammatory scarring alopecia: lichen planopilaris as the prototype

BACKGROUND: Lichen planopilaris (LPP) is the prototype of scarring alopecias that mainly target the infundibuloisthmic (bulge) region of hair follicle. Hair follicle stem cells have been shown to reside in the bulge. METHODS: We carried out this study to better define the possible pathogenetic role of the bulge in LPP. Thirty-five cases of LPP were studied. Multiple serial sections of biopsy specimens stained with hematoxylin and eosin, periodic acid Schiff-diastase, and Elastic van Gieson. The following immunostains were applied: CD3, CD4, CD8, CD1a, and Ki-67. Uninvolved follicles and normal scalp biopsy specimens served as normal controls. RESULTS: All cases showed a lichenoid lymphocytic infiltrate at the bulge region. The bulb area was spared. CD8(+) T cells were increased compared with CD4(+) T-cell population. Langerhans' cells were decreased. Proliferating stem cells, highlighted by Ki-67, showed a marked decrease in the bulge compared with uninvolved follicles. CONCLUSION: Our study supports the finding that in LPP, the inflammatory infiltrate mainly involves the bulge region, where the stem cells reside. Once this area is damaged, the hair loses its potential of regrowth with resulting scarring alopecia. This is in contrast with inflammatory non-scarring alopecias such as alopecia areata, where the bulb region is targeted, sparing the stem cells.