|Year : 2012 | Volume
| Issue : 1 | Page : 21-26
Clinico-pathological study of acquired primary cicatricial alopecias
CL Sowjanya1, T Narayana Rao1, P Guruprasad1, U Khopkar2
1 Department of DVL, Andhra Medical College, King George Hospital, Visakhapatnam, Andhra Pradesh, India
2 Department of Dermatology, Seth GS Medical College and KEM Hospital, Parel, Mumbai, India
|Date of Web Publication||21-Mar-2012|
T Narayana Rao
Professor of Dermatology, Department of DVL, King George Hospital, Visakhapatnam, Andhra Pradesh
Background: Alopecias are classified into non-scarring and scarring/cicatricial alopecias. Cicatricial alopecias represent a diverse group of diseases characterized by a lack of follicular ostia and irreversible alopecia. The main purpose of hair revolves around its profound role in social interactions. Loss of hair can lead to significant psychological and emotional distress. Hence, an accurate and early diagnosis and prompt intervention are necessary to prevent further follicular destruction.
Objective: To evaluate the epidemiological, clinical, and histopathological characteristics of various acquired primary cicatricial alopecias.
Materials and Methods: The study population consists of 50 patients with acquired primary cicatricial alopecias seen in our Dermatology Outpatient Department, during the period from September 2008 to September 2010.
Results: The most prevalent diagnoses in order of frequency lichen planopilaris (LPP, 50%), discoid lupus erythematosus (DLE, 20%), and pseudopelade of Brocq (POB, 12%). The ratio of lymphocytic to neutrophilic cicatricial alopecias was 7.3:1. A female predominance of lymphocytic cicatricial alopecias was observed. Both LPP and DLE affected predominantly middle-aged adults.
Conclusion: LPP and DLE followed by pseudopelade were the common cicatricial alopecias. In contrast to the previous large-scale studies, which showed DLE and/or pseudopelade as the foremost diagnosis, LPP is the most frequent cause of adult primary scarring alopecia in our study. An accurate diagnosis of cicatricial alopecia can be achieved only through careful clinicopathologic evaluation. Early scalp biopsy is mandatory in all cases. Further large-scale studies are necessary to establish a cause and pathogenesis behind this increased prevalence of LPP in our setting when compared to the western population.
Keywords: Primary scarring alopecias, lichen planopilaris, discoid lupus erythematosus, pseudopelade
|How to cite this article:|
Sowjanya C L, Rao T N, Guruprasad P, Khopkar U. Clinico-pathological study of acquired primary cicatricial alopecias. J Dr NTR Univ Health Sci 2012;1:21-6
|How to cite this URL:|
Sowjanya C L, Rao T N, Guruprasad P, Khopkar U. Clinico-pathological study of acquired primary cicatricial alopecias. J Dr NTR Univ Health Sci [serial online] 2012 [cited 2013 May 19];1:21-6. Available from: http://www.jdrntruhs.org/text.asp?2012/1/1/21/94171
| Introduction|| |
Cicatricial alopecias represent a diverse group of diseases characterized by a lack of follicular ostia and irreversible alopecia. ,, Both adults and children are affected. , It is usually circumscribed, but may be widespread. Exact pathogenesis is unknown. It is often multifactorial. A recent hypothesis suggests that in lichen planopilaris (LPP), the expression of peroxisome proliferator activated receptor gamma in hair follicle stem cell is significantly decreased.  Follicular destruction in scarring alopecia may be primary or secondary. In primary scarring alopecia, the hair follicle itself is the principal target for destruction, with relative sparing of the intervening dermis. Causes of primary scarring alopecia include discoid lupus erythematosus (DLE), LPP, pseudopelade, and folliculitis decalvans. Secondary scarring alopecia results from non-follicular events that impinge upon and eventually destroy the follicle. The permanent follicular scarring is the result of the close proximity of the follicles to the primary pathological process. The scarring may be secondary to numerous congenital or nevoid disorders affecting the hair follicle such as nevus sebaceous. Destruction of hair follicles may also result from physical and chemical trauma or pathological changes in the dermis because of sclerosing diseases or granulomatous or neoplastic infiltrates.
In many cases, extensive permanent damage is already present when patients are first seen, leaving little hope for regrowth with specific treatment. However, it should be possible to recognize and diagnose cases of scarring alopecia at an earlier stage in order to obtain better treatment outcomes. As the clinic-pathological data about primary scarring alopecias is infrequent, we examined their relative frequency and other epidemiological features, and evaluated their clinical and histopathological features.
| Materials and Methods|| |
Fifty patients with acquired primary cicatricial alopecia, with or without skin lesions, who attended our Dermatology Outpatient Department were evaluated. Complete history, including details of age, sex, occupation, symptoms, duration and course of the illness, was taken. A thorough clinical examination was done in all the patients. The affected area was biopsied under local anesthesia. A 4-mm punch biopsy was taken from the advancing edge of the scarring lesion and vertical sections were studied. In those cases which were to undergo direct immunofluorescence, the biopsy was taken both from lesional and perilesional areas.
| Observations and Results|| |
Patients were categorized into lymphocytic-mediated (DLE, LPP, and pseudopelade), neutrophilic-mediated (folliculitis decalvans), and mixed group (acne keloidalis nuchae) based on the NAHRS (North American Hair Research Society) working classification. The ratio of lymphocytic versus neutrophilic or mixed was 7.3:1. Our study included 50 patients of which 25 patients were affected with LPP (50%), 10 patients were affected with chronic cutaneous lupus erythematosus (CCLE) (20%), six patients had pseudopelade of Brocq (POB, 12%), keratosis follicularis spinulosa decalvans was noted in three patients (6%), folliculitis decalvans in three patients (6%), and acne keloidalis nuchae in three patients (6%).
Lichen planopilaris (LPP, 25 cases)
This category of cicatricial alopecia represented 50% of the total number of cases seen in our cohort. The female to male ratio was 1.8:1 (16 women and nine men). Age of the patients varied from 11 to 65 years with a mean age of onset of 38 years. The majority of patients (80.2%) were seen within 1 year of the onset of their clinical lesions. The four most common symptoms of LPP were increased shedding/hair loss (100%), pruritus (74%), scaling (56%), and scalp tenderness (28%). The majority of lesions presented as scaly hyperpigmented patches of alopecia, associated with atrophy. Characteristic findings were a positive pull for anagen hairs, perifollicular hyperpigmentation or erythema, hyperkeratotic papules, and scaling in active lesions. In a few cases, dilated follicular ostia, with loss of hair, were seen [Figure 1]a. Two patients presented with classical lesions of lichen planus on the scalp. The sites of involvement varied and there did not appear to be a predilection for a specific scalp site. Most of the patients presented with multifocal lesions. Four patients (16%) had cutaneous lesions of lichen planus, of which one had linear lichen planus. Nail changes like pitting, trachyonychia, longitudinal ridging, and dorsal pterygium, were documented in seven patients (28%).
|Figure 1: (a) Hyperpigmented papules, dilated follicular ostia, and loss of hair in lichen planopilaris (b) Histopathology of lichen planopilaris. (c) Immunofluorescence in lichen planopilaris|
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Histologic features consistent with LPP were follicular lymphocytic interface dermatitis, absence of sebaceous epithelium, and perifollicular lamellar fibrosis, with or without the presence of colloid bodies [Figure 1]b. In LPP, there was no increase in dermal mucin or subcutaneous inflammation. Direct immunofluorescence was done in five patients. Only one patient showed patchy deposition of IgM and C3 along the basement membrane zone [Figure 1]c. It was negative in four patients.
Discoid lupus erythematosus (DLE, 10 cases)
This represents 20% of the total number of cicatricial alopecia cases seen in our cohort. The female to male ratio was 4:1 (eight females and two males). The age of onset ranged from 17 to 57 years, with a mean age of 35.6 years. The majority of patients (89%) were seen within 1 year of onset of their clinical lesions. The most common symptoms were increased shedding/hair loss (100%), pruritus (35%), and scalp tenderness (46%). Salient clinical presentation consists of erythematous plaques of alopecia, atrophy, telangiectasia, depigmentation, and follicular hyperkeratosis [Figure 2]a. The activity of the scalp disease was reflected by a positive hair pull and prominent follicular hyperkeratosis, particularly in the centre of the plaque. Two patients (20%) with DLE had concomitant involvement of the other body sites.
|Figure 2: (a) Atrophy, depigmentation, and follicular hyperkeratosis in discoid lupus erythematosus (b) Histopathology of discoid lupus erythematosus|
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In all cases, histologic examination was consistent with the diagnosis of DLE. Characteristic histologic findings include epidermal atrophy with thickened basement membrane. Follicular vacuolar interface changes and perivascular and periadnexal dermal lymphocytic infiltrate are seen. Dermal mucin is present [Figure 2]b. Direct immunoflourescence (DIF) was done in one doubtful case and showed deposition of IgG along the dermoepidermal junction. Antinuclear antibodies were positive in two (20%) patients.
Pseudopelade of Brocq (POB, six cases)
This represents 12% of the total number of cicatricial alopecia cases seen in our cohort. The female to male ratio was 2:1. The characteristic clinical features were multiple, asymptomatic, 1-cm to 2-cm patches of cicatricial alopecia, resembling classical "footprints in snow" [Figure 3]a. The vertex and occipital scalp were involved in four cases. The remaining two cases showed multiple sites of involvement. The average duration to diagnosis was 3 years. Histologically, perifollicular lymphocytic infiltrate was reported with either a normal epidermis or an atrophic epidermis associated with absence of rete ridges. There was an absence of sebaceous glands and hair follicle. The end stage of pseudopelade was characterized by marked fibrosis and lack of inflammatory infiltrate, which was seen in four cases [Figure 3]b. The remaining two cases showed minimal perifollicular lymphocytic infiltrate.
|Figure 3: (a) Classical footprints in snow appearance in pseudopelade of Brocq (b) Histopathology of pseudopelade of Brocq|
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Keratosis follicularis spinulosa decalvans (KFSD, three cases)
Three cases (6%) presented with KFSD and all were females. Age of the patients varied from 7 years to 35 years with a mean age of 21 years. All three cases presented with patchy loss of hair and follicular papules, associated with minimal pruritus and scalp tenderness. There was no involvement of eyebrows or other body sites. None of the family members were affected with a similar problem. One patient presented with history of atopy. Histologic features were sparse superficial perivascular and peri-appendageal lymphocytic infiltrate with no epidermal change. Some of the follicular infundibula were dilated and plugged with orthokeratotic corneocytes. A few follicles in deep dermis showed dense perifollicular fibroplasia.
Folliculitis decalvans (FD, three cases)
FD was diagnosed in two females and one male. It presented as multiple follicular papules, pustules, and plaques [Figure 4]a. The lesions were multiple, painful, and were distributed over the entire scalp. Healing of the lesions left multiple 0.5-cm to 1-cm scars that sometimes resembled pseudopelade. Tufting of hairs was noted in one case. Swab cultures were positive for Staphylococcus aureus in two of the three cases. Histologic features included inflammatory accumulation of mostly neutrophils, with lymphocytes and plasma cells at varying depths [Figure 4]b. In quiescent stages, dermal and subcutaneous fibrosis was reported.
|Figure 4: (a) Pustules in folliculitis decalvans (b) Histopathology of folliculitis decalvans|
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Acne keloidalis nuchae (AKN, three cases)
Three patients presented with hypertrophic plaques and nodules on the occipital scalp and the nape of their neck [Figure 5]a. Few of the papules showed crusting. The lesions were associated with pruritus in all the three cases. All the three cases were reported in males (100%). Histologic features include perifollicular and intrafollicular lympho-plasmocytic infiltrate. Sebaceous glands were absent. Chronic inflammation, with numerous plasma cells and significant dermal fibrosis, was seen [Figure 5]b.
|Figure 5: (a) Papules and nodules on the occipital scalp and nape of neck in acne keloidalis nuchae (b) Histopathology of acne keloidalis nuchae|
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| Discussion|| |
There is paucity of data on cicatricial alopecia worldwide. , The inflammatory process predominantly occurs around the permanent portion of the hair follicle (stem cells of the bulge area and the infundibulum), hence resulting in the irreversibility of alopecia. , According to Whiting et al.,  pseudopelade was the commonest cause (40.6%), followed by LPP (12.6%) and FD (11.2%). Tan et al.,  reported DLE as the foremost cause(33.9%), followed by pseudopelade (24.1%) and LPP (22.3%). In contrast, in our study, LPP (50%) predominated. We reported a greater proportion of lymphocytic cicatricial alopecias versus neutrophilic cicatricial alopecias (7.3:1), which is in agreement with data reported by Tan et al.,  and also by Whiting (4:1). 
Lymphocytic cicatricial alopecias
LPP, CCLE, and POB were the three commonly occurring cicatricial alopecias. In accordance with previous studies, we observed a female predominance of lymphocytic cicatricial alopecias. ,,, In our study, both LPP and CCLE affected predominantly middle-aged adults in accordance with study by Tan et al.,  In LPP, the disease activity predominantly occurred at the periphery,  whereas in CCLE, the disease activity was primarily focused in the centre of the plaque. Scalp tenderness and pruritus were more pronounced in LPP. ,, A positive hair pull test assisted in determining the activity of disease. ,
Classic presentation of lichen planus is not observed on the scalp. ,,,, On the scalp, the classic clinical features include follicular hyperkeratotic papules, perifollicular erythema, and scaling at the periphery. Most of the patients presented with multifocal lesions. In our study, 16% of patients with LPP had cutaneous lesions of lichen planus, whereas it was 28% in a study conducted by Tan et al.  Nail changes like pitting, trachyonychia, longitudinal ridging, and dorsal pterygium, were documented in 28% of cases in our study. It was documented in 40% of cases by Tan et al.  Direct immunofluorescence was positive only in one (20%) of the five cases, whereas it was reported to be positive in 24% cases by Tan et al. 
CCLE of scalp may precede, coexist, or follow other skin lesions. In a study by Wilson et al.,  34% with CCLE had scalp involvement, and this may serve as a marker of chronicity and poor response to treatment. DLE lesions beyond scalp were noted in 25-41% in earlier studies. , In our study, 20% with CCLE had concomitant involvement of the other body sites.
The term "pseudopelade" is confusing. It denotes different meanings to different authors. ,,, Sperling et al.,  has attempted to redefine POB, follicular degeneration syndrome, and FD as subsets of central centrifugal scarring alopecia. In the NAHRS classification, classic POB and central centrifugal cicatricial alopecia belong to two distinct entities.  We considered pseudopelade as the classic reticulated "footprints in snow" pattern. The majority of pseudopelade behaves in an insidious manner and is rarely inflammatory. ,, Its noninflammatory nature frequently prevents early diagnosis.
The main treatment goal is to arrest the cicatrization process, decrease follicular inflammation, and halt further irreversible destruction. Treatment approaches are based on both the severity of disease and the patient's tolerance to treatment. Intralesional and potent topical steroids were prescribed in all active cases to reduce the inflammatory component while minimizing the systemic side effects. Short courses of oral steroids are helpful in controlling the acute inflammatory stages of LPP and CCLE. Antimalarials were used in CCLE and tetracyclines were tried in few cases of LPP. Hair transplantation was done, when the disease has been quiescent for at least 6 months.
Neutrophilic/mixed cicatricial alopecia
The pathogenesis is unknown. Staphylococcus aureus is usually cultured from the pustules, but it is unclear whether this is a primary or secondary process. The possible role of bacterial superantigens or a defect in cell-mediated immunity has been postulated. A female preponderance was noted in FD in contrast to the male predominance reported in previous studies. ,,, Acne keloidalis nuchae was reported predominantly in males.  A preferential involvement of the posterior half of the scalp was noted. Treatment given included systemic antibiotics and intralesional corticosteroids. CO 2 laser ablation was tried in few cases of acne keloidalis nuchae.
| Conclusion|| |
LPP, CCLE, and pseudopelade were the three commonly occurring cicatricial alopecias. We observed a female predominance in all lymphocytic cicatricial alopecias. LPP is a chronic condition and is the most frequent cause of adult primary scarring alopecia in our study. For proper management and better prognosis, biopsies should be taken in all cases of scarring alopecias as early as possible. This improves both, diagnostic ability and the chances of detecting cases early in the inflammatory stage before development of fibrosis, thus increasing the likelihood of a better response to treatment. Early diagnosis and timely therapeutic intervention are needed. Further research is needed on the pathogenesis, evaluation, and diagnosis of LPP, and randomized controlled trials are necessary to appreciate appropriate treatment.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]