Secondary cicatricial and other permanent alopecias

ABSTRACT:   Various nonfollicular scalp conditions can cause secondary scarring or permanent alopecia. Possible causes are congenital defects, trauma, inflammatory conditions, infections, and neoplasms (rarely drugs). Associated signs and symptoms and other diagnostic procedures such as histopathology may aid in the diagnosis. Detection of the underlying disorder may be difficult in end-stage lesions. Treatment is specific for active conditions. Surgery and hair transplantation are options for localized scars.     

Etiology of cicatricial alopecias: a basic science point of view

ABSTRACT:   This article presents a short summary of our current knowledge of cicatricial alopecia disease pathogenesis and the hypothetical disease mechanisms that may be involved in scarring alopecia development. Several forms of scarring alopecia likely involve targeted cytotoxic action against hair follicle cells mediated by a folliculocentric inflammation. However, the specific nature of the inflammatory interference in hair follicle growth is open to question. A popular hypothesis of lymphocyte-mediated scarring alopecia development involves autoimmune targeting of hair follicle–specific self-antigens, although there is no direct evidence in support of such a view. Alternative hypotheses focus on defects in sebaceous gland function, destruction of hair follicle stem cells, and interference in the communication between hair follicle mesenchyme and epithelium. Many questions arise from these hypotheses, and addressing them with a systematic research approach may enable significant advances in understanding cicatricial alopecia etiology.     

Update on primary cicatricial alopecias

The cicatricial alopecias encompass a diverse group of disorders characterized by permanent destruction of the hair follicle and irreversible hair loss. Destruction of the hair follicle can result from primary, folliculocentric disease or as a secondary result. This article focuses on the former, or primary cicatricial alopecias. The cause and pathogenesis of many of these disorders are largely unknown. Although unique clinicopathologic features allow for accurate diagnosis in some cases, diagnostic certainty is often elusive and reflects the limits of present understanding. Classification of the primary cicatricial alopecias on the basis of pathology provides a diagnostic and investigational framework and, it is hoped, will facilitate future enlightenment. Details of classification, etiopathogenesis, clinicopathologic features, differential diagnosis, and practical management of the primary cicatricial alopecias will be discussed. LEARNING OBJECTIVES: Upon completion of this learning activity, participants should be familiar with the following aspects of the primary cicatricial alopecias: (1) the new, consensus-issued classification scheme, (2) current understanding about etiopathogenesis, (3) salient clinicopathologic features, (4) differential diagnosis, and (5) therapeutic management.     

Psychosocial impact of cicatricial alopecias

Background:

Cicatricial alopecias have a significant impact on the psychological status, quality of life, and social interaction of those suffering from it. Till date, limited or no data have been available regarding the psychosocial and quality of life aspects of cicatricial alopecias.

Aims:

To assess the psychosocial impact of cicatricial alopecias.

Materials and Methods:

Thirty patients fulfilling the criteria for cicatricial alopecia irrespective of their age and sex were included in the study. Psychosocial assessment was carried out in 23 patients who were capable of responding to the questionnaire, using an adopted and suitably modified version of Women''s Androgenetic Alopecia Quality of Life Questionnaire.

Results:

We observed that 73.9% of our patients with cicatricial alopecias had moderate to severe psychosocial impact due to their hair loss. Patients of younger age group and with inactive disease, suffered from greater psychosocial impact of the disease. Patients with slight hair loss also had considerable psychological distress. The chronicity of disease duration did not seem to reduce the psychosocial impact of the disease. Both married and unmarried patients suffered equally from the psychosocial impact of the disease.

Conclusion:

The management of cicatricial alopecias needs a holistic approach. In addition to laying an emphasis on early diagnosis aided by clinco-pathological correlation, to prevent irreversible hair loss, the psychosocial impact of the disease should also be taken into consideration and addressed by the treating dermatologist.     

Trichoscopy of primary cicatricial alopecias: an updated review

Trichoscopy is an emerging technique for the evaluation of various hair and scalp disorders including primary cicatricial alopecias (PCAs). Early diagnosis of PCAs can lead to effective management and trichoscopy could be a useful aid during this process. Multiple studies have reported the trichoscopic features of PCAs, however, a focused review evaluating these disorders with their trichoscopic features is lacking. With the objective of evaluating the trichoscopic findings reported in the literature for various PCAs, we conducted a systematic literature search in PubMed and Embase. The search yielded 310 studies in a total of which 54 studies were included in our review. Majority of these studies originated from the Caucasian or Asian population. Through this review, we provide an overview of the frequent and specific trichoscopic findings in different PCAs with their clinical significance where applicable based on the current knowledge and identify the areas for future research.     

How not to get scar(r)ed: pointers to the correct diagnosis in patients with suspected primary cicatricial alopecia

Primary cicatricial alopecias (PCAs) are a rare, but important, group of disorders that cause irreversible damage to hair follicles resulting in scarring and permanent hair loss. They may also signify an underlying systemic disease. Thus, it is of paramount importance that clinicians who manage patients with hair loss are able to diagnose these disorders accurately. Unfortunately, PCAs are notoriously difficult conditions to diagnose and treat. The aim of this review is to present a rational and pragmatic guide to help clinicians in the professional assessment, investigation and diagnosis of patients with PCA. Illustrating typical clinical and histopathological presentations of key PCA entities we show how dermatoscopy can be profitably used for clinical diagnosis. Further, we advocate the search for loss of follicular ostia as a clinical hallmark of PCA, and suggest pragmatic strategies that allow rapid formulation of a working diagnosis.     

The surgical treatment of cicatricial alopecia

ABSTRACT:   Surgical correction of cicatricial alopecia can yield exceptional results when performed in the appropriate clinical scenario. To facilitate determination of the most suitable corrective therapy, we propose two new categories of cicatricial alopecia: "unstable" and "stable." Unstable cicatricial alopecia is intermittent and results in possible subsequent scarring hair loss in either new or old areas. Stable cicatricial alopecia, on the other hand, refers to fixed permanent scarring. While surgical excision is preferred to hair transplantation for both categories of cicatricial alopecia, this preference is even stronger in cases of unstable cicatricial alopecia due to its intermittent and progressive nature. Regardless of which corrective technique is used, analysis of specific physical patient characteristics coupled with a careful view towards the possible evolution of male pattern baldness or female pattern hair loss are essential to achieve superior long-term results. Herein we also outline guidelines for identifying these physical traits as well as for performing hair transplantation and surgical excision in order to achieve optimal cosmetic outcomes and minimize postoperative complications.     

Primary cicatricial alopecias: clinicopathology of 112 cases

BACKGROUND: Cicatricial alopecias represent a diverse group of diseases characterized by a lack of follicular ostia and irreversible alopecia. There is limited literature on the epidemiology and therapeutics of cicatricial alopecias. OBJECTIVE: The aim of this study was to review the epidemiology, clinical characteristics, and treatment of inflammatory cicatricial alopecias in a mixed ethnic population referred to a university hair clinic. METHODS: The study population consisted of 112 patients seen during a 5-year period with acquired primary cicatricial alopecias. This represented 3.2% of the total number of trichologic consultations seen at the University of British Columbia Hair Clinic, Vancouver, British Columbia, Canada. RESULTS: The ratio of lymphocytic to neutrophilic cicatricial alopecias was 4:1. Lymphocytic cicatricial alopecias had a tendency to affect middle-aged women, whereas neutrophilic cicatricial alopecias had a predilection for middle-aged men. CONCLUSIONS: An accurate diagnosis of cicatricial alopecia is achieved through careful clinicopathologic evaluation. We suggest that a scalp biopsy is mandatory in all cases. Multiple biopsies may be necessary for some affected individuals to achieve a definitive diagnosis as a result of a highly variable clinical course. An aggressive multiple modality therapeutic approach is often necessary to prevent further irreversible follicular destruction, implying cicatrical alopecia should be considered a trichologic emergency. Current therapeutic options for lymphocytic cicatricial alopecia include corticosteroids, antimalarials, and isotretinoin versus antibiotics, corticosteroids, and isotretinoin for neutrophilic cicatricial alopecias.     

Diagnostic accuracy measures for vertical and transverse scalp biopsies in cicatricial and non-cicatricial alopecias

Background

Scalp biopsy provides worthwhile diagnostic clues to diagnose the noncicatricial or cicatricial type of alopecia. Although a pair of vertically and horizontally sectioned pathology samples would be ideal, the diagnostic yield of vertical (V) or transverse (T) sectioning in different types of cicatricial and non-cicatricial alopecia is not studied. Also, when a single biopsy is submitted and/or the sample is not large enough for a combined V and T sectioning from a single specimen (such as HoVert technique), the decision to make the most appropriate sectioning would be challenging, specifically depending on the type of alopecia suspected clinically.

The histopathology of primary cicatricial alopecia

Alopecia typically is divided into cicatricial (scarring) and noncicatricial (nonscarring) forms. "Scarring" alopecia implies that follicular epithelium has been replaced by connective tissue and is therefore an irreversible process. In contrast, nonscarring alopecia is potentially reversible as follicular epithelia remain intact. Classification of cicatricial alopecia can be confusing and controversial as most disorders demonstrate overlapping clinical and histologic features. Herein, we present an overview of the histologic assessment of cicatricial alopecia, including an algorithmic approach to the evaluation of biopsy specimens from patients with scarring alopecia.     

Successful topical minoxidil treatment for hair density and length in trichorhinophalangeal syndrome type 1

A 9-year-old girl presented with slow hair growth and hair thinning since birth. Additionally, she had short stature and abnormally short fingers; genetic testing confirmed the diagnosis of trichorhinophalangeal syndrome (TRPS) type 1. After 4 months of topical minoxidil treatment, hair density and length significantly improved diffusely throughout the scalp without evidence of hypertrichosis. This case underscores the therapeutic potential of topical minoxidil for TRPS, paving the way for improved patient quality of life.     

Central centrifugal cicatricial alopecia

ABSTRACT:   A progressive scarring alopecia of the central scalp is commonly seen in young to middle-aged females of African descent. It usually starts at the vertex or mid top of the scalp and gradually spreads centrifugally, hence, the unifying term of central centrifugal cicatricial alopecia. The clinical pattern is suggestive of female pattern alopecia, but a lack of follicular pores indicative of scarring is present. It can progress for years before slowly burning out. The etiology is unknown but genetic factors may be important. It is often associated with a history of traumatic hairstyling involving heat, traction, and chemicals. However, most patients of African descent without this disorder have similar styling habits. Nonetheless, avoidance of physical and chemical trauma to the scalp hair, the use of suitable shampoos and conditioners, and the encouragement of natural hairstyles may be helpful. Any infection should be treated. Topical or intralesional corticosteroids and systemic antibiotics may be useful and topical minoxidil should be tried with the hope of preventing further scarring and encouraging regrowth of recovering follicles. Current research into the etiology of this disorder will help to foster much-needed clinical trials of therapeutic agents.     

毛发移植治疗瘢痕性秃发的研究进展

瘢痕性秃发是一组毛囊被结缔组织取代的疾病,分为原发性和继发性。原发性瘢痕性秃发病情隐匿反复,早期以控制原发病为主,晚期病情稳定可考虑外科修复,术后需长期随诊、药物维持治疗。继发性瘢痕性秃发多继发于烧伤、手术,瘢痕稳定后可考虑手术治疗。本文就毛发移植治疗瘢痕性秃发的研究进展进行综述。     

Medical and surgical therapies for alopecias in black women

Hair loss is a common problem that challenges the patient and clinician with a host of cosmetic, psychological and medical issues. Alopecia occurs in both men and women, and in all racial and ethnic populations, but the etiology varies considerably from group to group. In black women, many forms of alopecia are associated with hair-care practices (e.g., traction alopecia, trichorrhexis nodosa, and central centrifugal cicatricial alopecia). The use of thermal or chemical hair straightening, and hair braiding or weaving are examples of styling techniques that place African American women at high risk for various "traumatic" alopecias. Although the exact cause of these alopecias is unknown, a multifactorial etiology including both genetic and environmental factors is suspected. A careful history and physical examination, together with an acute sensitivity to the patient's perceptions (e.g., self-esteem and social problems), are critical in determining the best therapy course. Therapeutic options for these patients range from alteration of current hair grooming practices or products, to use of specific medical treatments, to hair replacement surgery. Since early intervention is often a key to preventing irreversible alopecia, the purpose of the present article is to educate the dermatologist on all aspects of therapy for hair loss in black women--including not only a discussion of the main medical and surgical therapies but also an overview of ethnic hair cosmetics, specific suggestions for alterations of hair-care practices, and recommendations for patient education and compliance.