Lichen planopilaris

Background

Lichen planopilaris (LPP) is an inflammatory condition of unknown etiology that affects pilosebaceous units, mainly of the scalp, and results in scaring alopecia.

Objective

A 51-year-old male presented with a pruritic eruption on the cheek consisting of atrophic macules and erythematous folliculocentric papules.

Results

Biopsy revealed a perifollicular lymphocytic infiltrate and vacuolar degeneration of the dermoepidermal junction consistent with LPP. Many treatment modalities have been utilized, with varying degrees of success. Our patient responded poorly to topical steroids. After nine months of topical tacrolimus therapy, his lesions resolved entirely.

Conclusion

The treatment of our patient demonstrates tacrolimus as a novel topical therapeutic option for patients with LPP.     

Lichen planopilaris

ABSTRACT:   Lichen planopilaris is a chronic scarring alopecia characterized by follicular hyperkeratosis, perifollicular erythema, and loss of follicular orifices. The scalp lesions may be single or multiple and commonly involve the vertex and parietal area. The hair follicles at the margin of the alopecic patches reveal perifollicular erythema. Anagen hairs can be pulled out easily in active lesions. Associated cutaneous, nail, and mucous membrane lichen planus may be present. Commonly encountered symptoms and signs are increased hair shedding, itching, scaling, burning, and tenderness. Differentiation from other cicatricial alopecia can be performed through meticulous evaluation of the clinical, histopathologic, and immunohistopathologic findings. Treatment strategies depend on the disease activity and physician expertise. Although there are no definitive curative modalities, some new discoveries and conceptual advances continue to broaden our treatment options of this complex condition.     

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: 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.     

Lichen planopilaris following hair transplantation and face-lift surgery

Cosmetic surgical procedures, including hair transplantation and face-lift surgery, are becoming increasingly popular. However, there is very little information regarding the associated development of dermatological conditions following these procedures. Lichen planopilaris (LPP) is an uncommon inflammatory hair disorder of unknown aetiology that results in permanent alopecia and replacement of hair follicles with scar-like fibrous tissue. Frontal fibrosing alopecia (FFA), a variant of LPP, involves the frontal hairline and shares similar histological findings with those of LPP. We report 10 patients who developed LPP/FFA following cosmetic scalp surgery. Seven patients developed LPP following hair transplantation, and three patients developed FFA following face-lift surgery. In all cases there was no previous history of LPP or FFA. There is currently a lack of evidence to link the procedures of hair transplantation and cosmetic face-lift surgery to LPP and FFA, respectively. This is the first case series to describe this connection and to postulate the possible pathological processes underlying the clinical observation. Explanations include Koebner phenomenon induced by surgical trauma, an autoimmune process targeting an (as yet, unknown) hair follicle antigen liberated during surgery or perhaps a postsurgery proinflammatory milieu inducing hair follicle immune privilege collapse and follicular damage in susceptible individuals.     

Lichen planopilaris: report of 30 cases and review of the literature

BACKGROUND: Lichen planopilaris (LPP) affects primarily the scalp, resulting in scaling, atrophy, and alopecia with scarring. The purpose of our study was to obtain original data on LPP and to evaluate the efficacy of topical therapy in comparison with systemic therapies. METHODS: We examined 30 patients affected by LPP between 1996 and 2001, performing clinical, laboratory, histopathologic and direct immunofluorescence examinations. Twenty-one of the patients (70%) were women and nine (30%) were men. The average age at presentation was 51.5 years. The average duration of the disease was 13 months at the time of the diagnosis. All patients received topical steroids for a total of 12 weeks. RESULTS: Resolution of the inflammatory process and blocking of the cicatricial progression were observed in 66% of cases, a mild reduction of fibrosis and cicatrization in 20% of patients, and no response in 13%. CONCLUSIONS: We concluded that topical therapy may be a valid alternative to systemic therapies, especially in patients with lesions in the early phase.     

Lichen planus sialadenitis: a mucosal analog of lichen planopilaris and lichen planoporitis

Lichen planus (LP) is the most prevalent dermatological disorder with oral manifestation. Oral lesions comprise a broad spectrum of clinical presentations. We report the case of a 56‐year‐old woman who presented erosive LP on the buccal and lower lip mucosae. Besides typical erosions, small white keratotic papules on an erythematosus background on the lower lip mucosa were observed. Biopsy of lower lip lesions showed an unusual histopathological presentation consisting of a lichenoid inflammation targeted to salivary gland ducts. This is probably a salivary gland analog of lichen planopilaris and lichen planoporitis. Lourenço SV, de Resende ACB, Bologna SB, Simonsen Nico MM. Lichen planus sialadenitis: a mucosal analog of lichen planopilaris and lichen planoporitis.     

Cutaneous lupus erythematosus and autoimmune thyroiditis

A 19-year-old girl presented with cutaneous lupus erythematosus and hypothyroidism, and developed a thyrotoxic crisis when chloroquine therapy was discontinued.     

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     

Dermatomyositis related to autoimmune thyroiditis

Background and objective Reports on the coexistence of dermatomyositis (DM) with autoimmune thyroiditis (AIT) are very few. Our aim is to define the relationship of the two conditions, identify clinical, laboratory, electromyographic and pathologic features of coexistent DM and AIT. Methods We underwent a MEDLINE search to identify relevant literature published in the past 30 years. Concurrently, we analysed retrospectively medical records of five patients diagnosed with DM and AIT from our hospital. Results Eleven cases were included. 90.9% of patients were female with a mean (SD) age of 44.18 (13.11) years for DM at diagnosis, and 39.00 (7.81) years for AIT. AIT can precede or parallel the diagnosis of DM. The most common comorbidities included hypothyroidism (90.9%), cardiopulmonary diseases (63.7%) and overlap syndrome (27.3%), while only one case had malignancy. The most common clinical manifestations were: muscle weakness (100%), polyarthralgia (45.5%), heliotrope rash (45.5%), myalgia (36.4%), and Raynaud’s phenomenon (27.3%). The abnormalities on electromyography and muscle/skin biopsy of DM related to AIT did not differ from those findings of DM, while none of these reports were normal. All patients received both the treatment of corticosteroids and levothyroxine, and only 27.3% of patients had a good prognosis. Conclusion Prevalence of cancer in coexistent DM and AIT may be very low. Also, it is reasonable to suggest that DM patients with AIT should be routinely evaluated for thyroid function and the emergence of comorbidities. Moreover, corticosteroids combined with levothyroxine may be useful for these patients as a standard treatment.     

Lichen planopilaris [cicatricial (scarring) alopecia] in a child.

A mother of a 12‐year‐old boy, 2 years ago, noticed that he showed patchy loss of hair on the vertex of the scalp. It was asymptomatic and progressive. Subsequently, similar patches appeared elsewhere on the scalp. Some of these patches joined to form a large bald patch. This was accompanied by dusky blue eruptions over the left upper lip and eyebrows. Later, there was localized loss of hair. A family history of a similar ailment was absent. Examination of the scalp revealed plaques of alopecia with mild to moderate erythema. The skin was smooth, shiny, and atrophic ( Fig. 1 ). Atrophy was apparent by the presence of wrinkles in places, and by holding the skin between the thumb and the index finger. The periphery of the lesions was well demarcated and was occupied by erythematous, scaly, follicular papules. Lesions were also located on the patches of alopecia. In addition, flat‐topped, dusky blue, papules/plaques were present over the upper lip.

Figure 1 Open in figure viewer PowerPoint Lichen planopilaris: plaques of alopecia showing smooth, shiny, atrophic skin with erythema     

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.