Cicatricial alopecia

In the classification of the North American Hair Research Society, primary cicatricial alopecias (PCA) are divided into four groups according to their prominent inflammatory infiltrate: PCAs with lymphocytic, neutrophilic, mixed or nonspecific cell inflammation pattern. The hair loss can begin subclinically and progress slowly so that the exact onset of the disease is often difficult to determine. The diagnosis is often delayed. While most forms of cicatricial alopecia can be clearly diagnosed based on clinical presentation in the acute disease stage, diagnosis can be challenging in the subacute, early or late disease stages. At first presentation, a detailed patient history and dermatological examination of the body, including trichoscopy, should be performed. In clinically unclear cases, a biopsy should be performed. Due to the scarcity of primary cicatricial alopecia, there is little evidence on the efficacy of the various therapies. The aims of treatment are to stop or at least delay hair loss and progression of the scarring process, reduce clinical inflammation signs as well as to alleviate subjective symptoms. Hair re‐growth in already scarred areas should not be expected. Anti‐inflammatory treatment with topical corticosteroids class III to IV and / or with intracutaneous intralesional triamcinolone acetonide injections can be considered in most of the primary cicatricial alopecias. The choice of systemic therapy depends on the type of predominant inflammatory infiltrate and includes antimicrobial, antibiotic or immunomodulating/immunosuppressive agents. Psychological support and camouflage techniques should be offered to the patients.     

Cicatricial alopecia due to sarcoidosis

Sarcoidosis of the scalp is a rare manifestation of cutaneous sarcoidosis. We report a case in a Syrian man without systemic involvement. Sarcoidosis should be considered in the differential diagnosis of cicatricial and non-cicatricial alopecia along with discoid lupus erythematous (DLE), lichen planopilaris, and scleroderma.     

Cicatricial alopecia: discoid lupus erythematosus

ABSTRACT:   Chronic cutaneous lupus erythematosus consists of three major dermatologic diseases: discoid lupus erythematosus (DLE), lupus panniculitis/lupus profundus, and lupus tumidus ( Table 1 ). DLE is estimated to be responsible for 50–85% of patients with chronic cutaneous lupus erythematosus. Scalp involvement is most often the presenting symptom. The clinical features and diagnosis of DLE, its pathophysiology and treatment, are reviewed.  

  Table 1.  Chronic cutaneous lupus erythematosus     

7.

Cicatricial alopecia following therapeutic embolization.     

L Toutous-Trellu  P Chavaz  P Piletta 《Acta dermato-venereologica》1999,79(3):236-237

     

8.

Cicatricial alopecia: classification and histopathology     

Somani N  Bergfeld WF 《Dermatologic therapy》2008,21(4):221-237

ABSTRACT:   Primary cicatricial alopecias are a diagnostically challenging group of disorders characterized by folliculocentric inflammation resulting in destruction of hair follicles and irreversible hair loss. They are classified according to a consensus-issued classification scheme based on the predominant cell type present: lymphocytic, neutrophilic, or mixed. Histopathology is a pivotal component of the diagnostic evaluation. Early diagnosis is critical since timely institution of treatment can halt progression of permanent hair loss. Salient histopathologic findings are presented in this review, along with adjunctive clues derived from interpretation of special stains and direct immunofluorescence studies. Despite careful evaluation, accurate diagnosis may remain elusive in some instances. The primary cicatricial alopecias often share overlapping features. The highest diagnostic yield is procured when histology is correlated with the clinical presentation.     

9.

Cicatricial alopecia: clinico-pathological findings and treatment     

Whiting DA 《Clinics in Dermatology》2001,19(2):211-225

     

10.

(25) Cicatricial alopecia? Cause     

Dr  R.C.D. Staughton Dr  G.C. Wells 《The British journal of dermatology》1974,91(S10):76-78

     

11.

Cicatricial alopecia occurring in two sisters from Ghana     

S.A.VAUGHAN JONES  M.M. BLACK 《Clinical and experimental dermatology》1994,19(6):500-502

We report two sisters from Ghana who presented with cicatricial alopecia simultaneously. In both cases the aetiology is unclear although both gave a history of previous scalp folliculitis. We classify the causes of cicatricial alopecia and the difficulties that can arise in placing such patients in a clinical category. Racial origin may be relevant in our cases; to our knowledge this is the first report of cicatricial alopecia occurring in two sisters.     

12.

Nonspecific diffuse alopecia as a single manifestation of syphilis infection: clinical and trichoscopic features     

《International journal of dermatology》2018,57(5):593-595

     

13.

Cicatricial alopecia of the scalp with histological aspect of syringoma]     

G Tsoitis  C Asvesti  C Mandinaos  M Lambroudi  J Hatzibougias  A Babi 《Annales de dermatologie et de vénéréologie》1992,119(11):919-922

     

14.

以弥漫性秃发为首发表现的亲毛囊性蕈样肉芽肿一例          下载免费PDF全文

暴芳芳  付希安  孙乐乐  刘永霞  周桂芝  刘红 《中国麻风皮肤病杂志》2019,35(1):36-38

亲毛囊性蕈样肉芽肿属于蕈样肉芽肿的一种亚型，侵袭性较强，预后差，早期诊断尤为重要。本文报道一例免疫组化联合TCR基因重排确诊的以弥漫性秃发为首发表现亲毛囊性蕈样肉芽肿一例。     

15.

Segmental manifestation: a clue to explain the nature of pigmented purpuric dermatoses     

Torchio D 《The Australasian journal of dermatology》2011,52(3):235-235

     

16.

Circumscribed alopecia: an unusual manifestation of pediculosis capitis     

Hall RR  McMichael AJ 《Pediatric dermatology》2012,29(4):513-514

A healthy 10-year-old girl was referred for evaluation of patchy hair loss on the scalp of longer than 6 months duration. She had been diagnosed and treated for head lice approximately 1 month before onset of the hair loss. Examination of the scalp showed discrete ovoid patches of hair loss at the vertex scalp. A scrape of the area of hair loss was performed, and a nit was visible on microscopic examination. Focal hair loss may represent an atypical manifestation of ongoing pediculosis capitis.     

17.

Cicatricial alopecia secondary to radiation therapy: case report and review of the literature     

Severs GA  Griffin T  Werner-Wasik M 《Cutis; cutaneous medicine for the practitioner》2008,81(2):147-153

Cicatricial or scarring alopecia represents permanent destruction of the hair follicle, histopathologically showing a decreased number of follicular units leaving streamers of fibrosis or hyalinization of surrounding collagen. High-dose radiation therapy (RT) used for treating intracranial malignancy can permanently destroy hair follicles, resulting in permanent alopecia. Typically, there also is clinical scarring of the skin with dermal fibrosis. We report a case of radiation-induced cicatricial alopecia confirmed by histopathology, without obvious clinical scarring or dermal fibrosis. This lack of fibrosis made our patient a good candidate for hair transplantation. The clinicopathologic presentation in this case could be related to the method of RT employed in treating our patient's brain tumor. A literature review of radiation-induced cicatricial alopecia, as well as a brief discussion of the current radiation methods used in the treatment of intracranial malignancy, is presented. We believe that because most anagen follicles are approximately 4 mm deep in the skin, if the dose of radiation superficial to a depth of 5 mm is kept under 16 Gy, which is the approximate lethal dose for hair follicles, the incidence of radiation-induced cicatricial alopecia could be avoided or markedly decreased.     

18.

Psoriatic alopecia manifestation, course and therapy in 34 patients     

U Runne  P Kroneisen 《Zeitschrift für Hautkrankheiten》1989,64(4):302-4, 307-10, 313-4

We report on a long-term clinical study on 34 patients (aged 6 to 88) suffering from psoriasis vulgaris capillitii with ensuing loss of hair and alopecia. Most patients showed heavy hyperkeratosis. The loss of hair was either massive (sometimes in tufts) or moderate; its course was acute in 53%, chronically recurrent in 15%, and chronically progressive in 32% of the cases. The psoriatic alopecia was circumscribed in 80% (from the size of a coin up to that of a palm of a hand; thinned out or bare) and diffuse in 20% of the patients. Alopecia was only observed in psoriatic lesions, but not necessarily in every plaque. Thus, psoriasis of the entire scalp did either result in circumscribed or in diffuse alopecia. The trichogram taken from regions next to alopecic areas revealed a telogen rate between 25 and 86%, according to the respective progression of the disease. The light microscope showed the typical features of psoriasis associated with distinct follicular hyper-(para-)keratosis; in some of the histologic sections, we saw a well-defined perifollicular adnexophilic infiltration of lymphocytes and histiocytes, which could cause alterations of the follicular epithelium and sometimes even destroy it altogether. In due course, these alterations resulted in granulomatous foreign-body reaction with scar formation. After topical anti-psoriatic treatment, we noticed regrowth of hair in more than 70% of the patients, whereas 25% developed scarring alopecia. - What we call psoriatic alopecia is the symptomatic loss of hair in psoriatic lesions of the scalp. In most of the cases, it can easily be identified on account of the underlying psoriasis.(ABSTRACT TRUNCATED AT 250 WORDS)     

19.

Cicatricial alopecia; a dermatopathologic and immunopathologic study of 33 patients (pseudopelade of Brocq is not a specific clinico-pathologic entity)     

Amato L  Mei S  Massi D  Gallerani I  Fabbri P 《International journal of dermatology》2002,41(1):8-15

BACKGROUND: Pseudopelade of Brocq (PB) is a permanent progressive scarring alopecia characterized by numerous alopecic patches localized only in the scalp, that tend to coalesce into larger, irregular plaques with policyclic borders. PB can be considered either the final atrophic stage of several scarring disorders such as lichen planus pilaris (LPP) and discoid lupus erythematosus (DLE) (secondary PB) or an autonomous disease (primary PB). The aim of this study was to assess the incidence of primary vs. secondary PB by a combined histopathological and immunopathological study in a series of patients who fulfilled the clinical diagnostic criteria for PB set forth by Braun Falco et al. METHODS: We studied 33 patients (5 males and 28 females, whose age ranged from 24 to 75 years). The duration of the disease (from onset to biopsy) ranged from 3 months to 8 years. Serum samples were tested for circulating auto-antibodies (antinuclear antibodies anti ENA, anticentromere, anti-Scl70, antithyroid, antigastric parietal cells) circulating immune complexes, total and single fraction (C3, C4) complement activity. The skin biopsies taken from the active advancing margin of the more recent alopecic patch were bisected vertically, one was sent for histopathological examination, and the other for the immunofluorescence studies. RESULTS: In all patients the serum tests above were found to be negative or normal. Histopathologically, 11 biopsies (33.3%) displayed findings typical for LPP whereas seven cases (21.2%) showed typical DLE features. In the remaining 15 cases (45.5%) histopathological findings were not suggestive of any specific dermatosis. DIF investigations showed findings typical of LPP in six cases (18.1%) and typical of DLE in seven cases (21%). In three cases we did not find findings typical of LPP, DLE, or any other specific dermatitis. In 11 cases no immunological deposits could be detected and therefore were classified as negative. CONCLUSION: In conclusion, PB is a type of scarring alopecia of the scalp associated with a peculiar clinical presentation and evolution, which cannot be considered an autonomous nosologic entity because in 66.6% of patients it is the end stage of other inflammatory chronic diseases such as LPP and DLE. It is conceivable that even in those cases in which the histopathological and immunopathological findings did not allow for a specific diagnosis, LPP and DLE were also involved. It is noteworthy that in our study the histopathological and the immunopathological examinations did not conflict and often the results were even coincidental, thus confirming the compatibility of the combined histo-immunopathological approach in the diagnostic evaluation of PB.     

20.

Erythroderma as a manifestation of leprosy     

《Anais brasileiros de dermatologia》2019,94(1):89-92

     

Cicatricial alopecia: classification and histopathology

ABSTRACT:   Primary cicatricial alopecias are a diagnostically challenging group of disorders characterized by folliculocentric inflammation resulting in destruction of hair follicles and irreversible hair loss. They are classified according to a consensus-issued classification scheme based on the predominant cell type present: lymphocytic, neutrophilic, or mixed. Histopathology is a pivotal component of the diagnostic evaluation. Early diagnosis is critical since timely institution of treatment can halt progression of permanent hair loss. Salient histopathologic findings are presented in this review, along with adjunctive clues derived from interpretation of special stains and direct immunofluorescence studies. Despite careful evaluation, accurate diagnosis may remain elusive in some instances. The primary cicatricial alopecias often share overlapping features. The highest diagnostic yield is procured when histology is correlated with the clinical presentation.     

Cicatricial alopecia occurring in two sisters from Ghana

We report two sisters from Ghana who presented with cicatricial alopecia simultaneously. In both cases the aetiology is unclear although both gave a history of previous scalp folliculitis. We classify the causes of cicatricial alopecia and the difficulties that can arise in placing such patients in a clinical category. Racial origin may be relevant in our cases; to our knowledge this is the first report of cicatricial alopecia occurring in two sisters.     

以弥漫性秃发为首发表现的亲毛囊性蕈样肉芽肿一例

亲毛囊性蕈样肉芽肿属于蕈样肉芽肿的一种亚型，侵袭性较强，预后差，早期诊断尤为重要。本文报道一例免疫组化联合TCR基因重排确诊的以弥漫性秃发为首发表现亲毛囊性蕈样肉芽肿一例。     

Circumscribed alopecia: an unusual manifestation of pediculosis capitis

A healthy 10-year-old girl was referred for evaluation of patchy hair loss on the scalp of longer than 6 months duration. She had been diagnosed and treated for head lice approximately 1 month before onset of the hair loss. Examination of the scalp showed discrete ovoid patches of hair loss at the vertex scalp. A scrape of the area of hair loss was performed, and a nit was visible on microscopic examination. Focal hair loss may represent an atypical manifestation of ongoing pediculosis capitis.     

Cicatricial alopecia secondary to radiation therapy: case report and review of the literature

Cicatricial or scarring alopecia represents permanent destruction of the hair follicle, histopathologically showing a decreased number of follicular units leaving streamers of fibrosis or hyalinization of surrounding collagen. High-dose radiation therapy (RT) used for treating intracranial malignancy can permanently destroy hair follicles, resulting in permanent alopecia. Typically, there also is clinical scarring of the skin with dermal fibrosis. We report a case of radiation-induced cicatricial alopecia confirmed by histopathology, without obvious clinical scarring or dermal fibrosis. This lack of fibrosis made our patient a good candidate for hair transplantation. The clinicopathologic presentation in this case could be related to the method of RT employed in treating our patient's brain tumor. A literature review of radiation-induced cicatricial alopecia, as well as a brief discussion of the current radiation methods used in the treatment of intracranial malignancy, is presented. We believe that because most anagen follicles are approximately 4 mm deep in the skin, if the dose of radiation superficial to a depth of 5 mm is kept under 16 Gy, which is the approximate lethal dose for hair follicles, the incidence of radiation-induced cicatricial alopecia could be avoided or markedly decreased.     

Psoriatic alopecia manifestation, course and therapy in 34 patients

We report on a long-term clinical study on 34 patients (aged 6 to 88) suffering from psoriasis vulgaris capillitii with ensuing loss of hair and alopecia. Most patients showed heavy hyperkeratosis. The loss of hair was either massive (sometimes in tufts) or moderate; its course was acute in 53%, chronically recurrent in 15%, and chronically progressive in 32% of the cases. The psoriatic alopecia was circumscribed in 80% (from the size of a coin up to that of a palm of a hand; thinned out or bare) and diffuse in 20% of the patients. Alopecia was only observed in psoriatic lesions, but not necessarily in every plaque. Thus, psoriasis of the entire scalp did either result in circumscribed or in diffuse alopecia. The trichogram taken from regions next to alopecic areas revealed a telogen rate between 25 and 86%, according to the respective progression of the disease. The light microscope showed the typical features of psoriasis associated with distinct follicular hyper-(para-)keratosis; in some of the histologic sections, we saw a well-defined perifollicular adnexophilic infiltration of lymphocytes and histiocytes, which could cause alterations of the follicular epithelium and sometimes even destroy it altogether. In due course, these alterations resulted in granulomatous foreign-body reaction with scar formation. After topical anti-psoriatic treatment, we noticed regrowth of hair in more than 70% of the patients, whereas 25% developed scarring alopecia. - What we call psoriatic alopecia is the symptomatic loss of hair in psoriatic lesions of the scalp. In most of the cases, it can easily be identified on account of the underlying psoriasis.(ABSTRACT TRUNCATED AT 250 WORDS)     

Cicatricial alopecia; a dermatopathologic and immunopathologic study of 33 patients (pseudopelade of Brocq is not a specific clinico-pathologic entity)

BACKGROUND: Pseudopelade of Brocq (PB) is a permanent progressive scarring alopecia characterized by numerous alopecic patches localized only in the scalp, that tend to coalesce into larger, irregular plaques with policyclic borders. PB can be considered either the final atrophic stage of several scarring disorders such as lichen planus pilaris (LPP) and discoid lupus erythematosus (DLE) (secondary PB) or an autonomous disease (primary PB). The aim of this study was to assess the incidence of primary vs. secondary PB by a combined histopathological and immunopathological study in a series of patients who fulfilled the clinical diagnostic criteria for PB set forth by Braun Falco et al. METHODS: We studied 33 patients (5 males and 28 females, whose age ranged from 24 to 75 years). The duration of the disease (from onset to biopsy) ranged from 3 months to 8 years. Serum samples were tested for circulating auto-antibodies (antinuclear antibodies anti ENA, anticentromere, anti-Scl70, antithyroid, antigastric parietal cells) circulating immune complexes, total and single fraction (C3, C4) complement activity. The skin biopsies taken from the active advancing margin of the more recent alopecic patch were bisected vertically, one was sent for histopathological examination, and the other for the immunofluorescence studies. RESULTS: In all patients the serum tests above were found to be negative or normal. Histopathologically, 11 biopsies (33.3%) displayed findings typical for LPP whereas seven cases (21.2%) showed typical DLE features. In the remaining 15 cases (45.5%) histopathological findings were not suggestive of any specific dermatosis. DIF investigations showed findings typical of LPP in six cases (18.1%) and typical of DLE in seven cases (21%). In three cases we did not find findings typical of LPP, DLE, or any other specific dermatitis. In 11 cases no immunological deposits could be detected and therefore were classified as negative. CONCLUSION: In conclusion, PB is a type of scarring alopecia of the scalp associated with a peculiar clinical presentation and evolution, which cannot be considered an autonomous nosologic entity because in 66.6% of patients it is the end stage of other inflammatory chronic diseases such as LPP and DLE. It is conceivable that even in those cases in which the histopathological and immunopathological findings did not allow for a specific diagnosis, LPP and DLE were also involved. It is noteworthy that in our study the histopathological and the immunopathological examinations did not conflict and often the results were even coincidental, thus confirming the compatibility of the combined histo-immunopathological approach in the diagnostic evaluation of PB.