Centrifugal lipodystrophy of the scalp presenting with an arch-form alopecia: A 10-year follow-up observation

We describe a 10-year follow-up observation of progressive arch-form alopecia caused by centrifugal lipodystrophy (CLD) in a Japanese boy. A 2.5-year-old boy developed a slightly depressed lesion demarcated by a horseshoe-shaped erythematous border on his right neck, which then extended to the scalp. Four years later, arch-form alopecia became apparent in the right temporal region along with an erythematous border. The arch-form alopecia gradually expanded centrifugally, leaving a slight residual depression, but hair regrowth was seen within the area of alopecia. Histological examination of the erythematous border revealed non-specific inflammatory changes in the subcutaneous fat. Magnetic resonance imaging findings revealed a loss of subcutaneous fat inside the lesion. The alopecia continuously extended until he was 12 years old, but, thereafter, expansion ceased and hair regrowth gradually occurred in the arch-form alopecia. A linear non-hairy lesion 5 cm in length still remained when he was 13 years old. CLD might involve the scalp and cause linear, arch-form alopecia.     

Female pattern hair loss and its relationship to permanent/cicatricial alopecia: a new perspective

Female pattern hair loss (FPHL) is a common hair disorder of the central scalp. The clinical change in hair density, related to a change in the hair cycle and miniaturization of the hair follicle, is generally considered to be potentially reversible. However, there is now evidence of a permanent hair loss that develops in a subset of women with FPHL. The presence of a perifollicular lymphohistiocytic infiltrate and fibrosis is seen without follicular drop-out in biopsies of women with FPHL and with a notable follicular drop-out in a cicatricial form of this condition (heretofore called cicatricial pattern hair loss) as well as in fibrosing alopecia in a pattern distribution, currently classified as a subset of lichen planopilaris. The potential relationship of these conditions as well as frontal fibrosing alopecia and central centrifugal cicatricial alopecia, two other conditions of permanent hair loss seen primary in women, is discussed.     

Central centrifugal cicatricial alopecia - an approach to diagnosis and management

Central centrifugal cicatricial alopecia (CCCA) occurs primarily in African-American women and is the most common cause of scarring hair loss in this population. Since the mid 20th century, hair care practices of African-American women have been associated with CCCA, although there is developing evidence that the etiology of CCCA may be multifactorial. Clinically diagnosing CCCA may be challenging because it can resemble female pattern hair loss, alopecia areata, lichen planopilaris, or telogen effluvium. Therapeutic options are limited, thus the goal of treatment is to prevent progression of disease because once scar formation occurs, it is irreversible.     

Prospective histologic examinations in patients who practice traumatic hairstyling

Objectives Alopecia is the fifth most common dermatologic diagnosis in African‐American patients. Central centrifugal cicatricial alopecia (CCCA) is the most common form of scarring alopecia in this group. This study sought to evaluate clinical and histologic findings in patients without clinical alopecia who use chemical and/or thermal straighteners to determine whether follicular damage is evidenced histologically. Methods Eight African‐American women with no clinical evidence of alopecia or scalp inflammation were included in the study. All participants had engaged in some form of traumatic hair care within the previous month. Participants submitted to clinical photography and 4‐mm punch biopsy. Histologic examination was performed and the characteristics of each case recorded. Results There were no clinical signs of alopecia or inflammation in any patient. Histopathology showed peri‐infundibular lymphocytic inflammation in all patients and mild superficial perivascular lymphocytic inflammation in three. Concentric infundibular fibrosis was observed in each hair follicle in all specimens. One sample showed additional focal peri‐isthmus fibrosis. There was no evidence of complete follicular dropout, follicular epithelial thinning, or premature desquamation of inner root sheaths. The mean number of hair follicles was 4.88 per 4‐mm punch. Hair cycling was consistently within normal ranges. Conclusions Biopsy findings characteristic of CCCA suggest that a clinical prelude exists histologically. Further follow‐up may provide a longitudinal timeframe for the potential progression, halting, or reversal of disease if hairstyling practices are, respectively, continued or discontinued. Central centrifugal cicatricial alopecia is likely to represent a common pathway of inflammation and scarring that can be instigated by traumatic hairstyling practices in genetically predisposed subjects.     

Centrifugal lipodystrophy of the scalp manifesting as centrifugal lipodystrophic alopecia

Centrifugal lipodystrophy (CLD), characterized by a depressed lesion in the abdominal skin, is a chronic disease occurring more often among younger patients of East Asian descent. We present an extremely unusual case of CLD of the scalp associated with reversible hair loss. The patient demonstrated alopecia in the frontal, temporal and occipital areas of the scalp, which connected to form a ring‐shaped area of hair loss. Curiously, the area of hair loss gradually expanded outwards while the central region showed normal hair regrowth. Immunohistochemical analysis demonstrated reduced expression of leptin, an adipokine capable of inducing the anagen phase of the hair cycle, in the adipose tissue, associated with active inflammation. By contrast, recovery of leptin expression was observed at sites of healed inflammatory lesions, suggesting that reversible hair loss might be caused by a change in leptin expression in adipose tissue.     

'A detective look' at hair biopsies from African-American patients

Background A patient’s ethnicity can be an important clue in the diagnosis of scarring alopecia as some disorders such as traction alopecia (TA) and central centrifugal cicatricial alopecia (CCCA) are more prevalent in or exclusive to African‐Americans. Objectives To perform a retrospective review of 60 scalp biopsies from African‐American patients including 25 cases of CCCA, 22 cases of TA, five cases of frontal fibrosing alopecia, three cases of discoid lupus erythematosus, three cases of hair breakage and two cases of alopecia areata. Methods Serial horizontal and vertical sections were examined. Results Features characteristic of the African‐American scalp include: golf club‐shaped bulb, elliptical shape of the hair shaft, asymmetrical outer root sheath and paired grouping of hair follicles. Clues to the diagnosis of CCCA include: premature desquamation of the inner root sheath, goggles and naked hair shafts in fibrous streamers. Diagnosis of TA is suggested by preserved sebaceous glands along with follicular miniaturization and drop‐out. Conclusions The clues reported here aim to help the dermatopathologists to: recognize at a glance that they are dealing with a scalp biopsy from an African‐American patient; make the most probable diagnosis by connecting the clues (even if only vertical sections are present); and understand the morphological basis for the susceptibility of the African hair to damage.     

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.     

Centrifugal lipodystrophy of the face mimicking progressive lipodystrophy

Summary We report a Japanese boy with centrifugal lipodystrophy. The initial lesion developed on the forehead as an area of erythema which showed centrifugal spread and central fading to leave a residual depression. Three years later, similar erythematous lesions appeared on the cheeks, and he developed a cadaverous appearance. A central depression on the upper trunk was surrounded by an erythematous border and there was right cervical lymphadenopathy. Histological examination revealed inflammatory changes in the subcutaneous fat. Although this patient showed the characteristics of both centrifugal and progressive lipodystrophy (partial lipodystrophy), we consider that he has centrifugal lipodystrophy of the face because of the overall features of the lesions.     

Central scalp alopecia photographic scale in African American women

ABSTRACT:   Central centrifugal cicatricial alopecia (CCCA) is a common but poorly understood cause of hair loss in African American women. A photographic scale was developed that captures the pattern and severity of the central hair loss seen with CCCA in order to help identify this problem in the general community and to potentially correlate clinical data with hair loss. The utility and reproducibility of this photographic scale was determined in a group of 150 African American women gathered for a health and beauty day who were evaluated by both four investigators experienced in the diagnosis of hair disorders and by the subjects themselves.     

Characterization of the inflammatory features of central centrifugal cicatricial alopecia

Central centrifugal cicatricial alopecia (CCCA) is a scarring alopecia that primarily affects women of African descent. Although histopathological features of CCCA have been described, the pathophysiology of this disease remains unclear. To better understand the components of CCCA pathophysiology, we evaluated the composition of the inflammatory infiltrate, the distribution of Langerhans cells (LCs), and the relationship between fibrosis and perifollicular vessel distribution. Our data indicate that CCCA is associated with a CD4-predominant T-cell infiltrate with increased LCs extending into the lower hair follicle. Fibroplasia associated with follicular scarring displaces blood vessels away from the outer root sheath epithelium. These data indicate that CCCA is an inflammatory scarring alopecia with unique pathophysiologic features that differentiate it from other lymphocytic scarring processes.     

Cytokeratin 15 expression in central, centrifugal, cicatricial alopecia: new observations in normal and diseased hair follicles

Background: Cytokeratin 15 (CK15) is a useful marker for the bulge zone (BZ) and has been used to examine follicles in cicatricial alopecia. We studied the expression of CK15 in hair follicles of patients with central, centrifugal, cicatricial alopecia (CCCA) in an attempt to define BZ integrity. Methods: A commercially available antibody to CK15 was used on formalin‐fixed, paraffin‐embedded tissue from clinically and histologically ‘normal’ scalps, clinically diseased scalps from patients with CCCA and clinically ‘normal’ scalps from patients with CCCA. Results: In both normal and diseased follicles, CK15 expression was closely linked to anatomical zone cellular morphology. Normal and abnormal inner root sheath (IRS) desquamation occurred in concert with predictable cellular morphological changes and CK15 expression. In most abnormal follicles, once the IRS desquamated, the morphology of BZ epithelium changed and CK15 expression disappeared. Conclusions: CK15 highlights BZ cells in normal human follicles, but may be unreliable for this purpose in diseased follicles. CK15 should not be the sole marker for studying stem cells in cicatricial alopecia because any disease‐induced structural changes could alter CK15 expression. More sophisticated studies of stem cells will be required to reliably define their role in the pathogenesis of cicatricial alopecia. Sperling LC, Hussey S, Wang J, Darling T. Cytokeratin 15 expression in central, centrifugal, cicatricial alopecia: new observations in normal and diseased hair follicles.     

Dermoscopy guided scalp biopsy in cicatricial alopecia

Background Scalp biopsies are crucial for the diagnosis of cicatricial alopecia. However, the pathologic interpretation may not be diagnostic if biopsy is not obtained from the correct site. This is particularly relevant for cicatricial alopecia as the disease may be focal and disease activity difficult to appreciate by the naked eye. Objective To report a new simple technique to select the optimal biopsy site in cicatricial alopecia. Methods In the last 2 years we performed dermoscopy guided scalp biopsies using handled dermatoscopes in 80 patients with different forms of cicatricial alopecia. Biopsy site was selected based on presence of the following dermatoscopic features: perifollicular concentric white scales in lichen planopilaris, frontal fibrosing alopecia (FFA) and discoid lupus erythematosus (DLE); hair tufts in folliculitis decalvans, hairs surrounded by a peripilar grey‐white halo in central centrifugal cicatricial alopecia and follicular red dots or keratotic plugs in DLE. Results The dermoscopy guided biopsies yielded a definitive pathological diagnosis in 95% of the cases. Comment The advantage of this method is that it is a fast, precise way to identify even individually affected follicles in early or focal cicatricial alopecia. It also allows for the morphologic characterization of particular follicular structures.     

Follicular and Scarring Disorders in Skin of Color: Presentation and Management

Skin of color, also known as ethnic skin, is described as skin of individuals of African, Asian, Hispanic, Native-American, Middle Eastern, and Pacific Island backgrounds. Differences in hair morphology, hair grooming, cultural practices, and susceptibility to keloid scarring exist within these populations and have been implicated in hair, scalp, and skin disorders. Acne keloidalis (AK), central centrifugal cicatricial alopecia (CCCA), dissecting cellulitis of the scalp (DCS), pseudofolliculitis barbae (PFB), traction alopecia (TA), and keloids are the most prevalent follicular and scarring disorders in skin of color. They have been associated with disfigurement, permanent hair loss, emotional distress, and decreased quality of life. Hair grooming practices, such as the use of chemical relaxers, heat straightening, and tight braiding and weaving can cause scalp irritation and follicular damage and are linked to the pathogenesis of some of these conditions. Consequently, patient education and behavior modifications are integral to the prevention and management of these disorders. Scarring disorders are also of concern in ethnic populations. Keloid scarring is more prevalent in individuals of African, Asian, and Hispanic descent. The scarring alopecia CCCA is almost exclusively seen in patients of African descent. Therapeutic regimens such as intralesional corticosteroids, surgical excision, and laser therapy can be effective for these follicular and scarring disorders, but carry a risk of dyspigmentation and keloid scarring. Ethnic skin and hair may present unique challenges to the clinician, and knowledge of these differences is essential to providing quality care.     

Prevalence of cutaneous adverse effects of hairdressing: a systematic review

OBJECTIVE: To identify studies of the prevalence of cutaneous complications of hairdressing in (1) hairdressers and the general population and (2) those more common in people of African ancestry. DATA SOURCES: Three versions of MEDLINE were searched from January 1966 through December 2004 and with a repeated search in August 2005 using 2 groups of search terms: group 1, terms used for hair care and specific study designs: survey, cross-sectional study, and cohort study; group 2, the terms African hair, Afro-Caribbean hair, African American hair, central centrifugal cicatricial alopecia, acne keloidalis nuchae, traction alopecia, and synonyms for each. STUDY SELECTION AND DATA EXTRACTION: All identified cross-sectional and cohort studies of cutaneous adverse effects were included and their quality assessed using criteria developed by Radulescu et al. DATA SYNTHESIS: Four studies used either questionnaires or patch testing to estimate the prevalence of cutaneous adverse effects of hair chemicals in the general population and found a prevalence of contact dermatitis, secondary to use of hair dye, of 5.3% and of allergy to paraphenylenediamine of 0.1% to 2.3%. Working as a hairdresser is associated with a prevalence of contact dermatitis ranging from 16.4% in larger cohort studies that included a clinical examination to 80% in the smaller, questionnaire-based studies. Three studies of people of African ancestry found a prevalence of acne keloidalis nuchae ranging from 1.3% to 13.7% and of traction alopecia of 1%. None of these were in the general population. CONCLUSIONS: Working as a hairdresser is associated with an increased risk of contact allergy and/or hand dermatitis. Studies of skin disorders of individuals of African ancestry are needed to quantify the health burden and clarify causal variables of these disorders. It is not clear how much the unique shape of the African hair follicle contributes to the development of these conditions.     

Cytokeratin 75 expression in central,centrifugal, cicatricial alopecia – new observations in normal and diseased hair follicles

Background: Premature desquamation of the inner root sheath (IRS) is an important histological marker for central, centrifugal, cicatricial alopecia (CCCA), and an inherently defective IRS may be responsible. Cytokeratin 75 (K75; formerly K6hf) is an appealing candidate for study because K75 is specifically expressed in the companion layer of the hair follicle, the interface for IRS desquamation. Also, K75 abnormalities have been found in other hair diseases bearing similarities to CCCA. Methods: We used a commercially available antibody to K75 on formalin‐fixed, paraffin‐embedded tissue from clinically and histologically “normal” scalp (n=9); clinically diseased scalp from patients with CCCA (n=15); and clinically “normal” scalp from patients with CCCA (n=6). Results: K75 expression disappears during the process of IRS desquamation, and loss of expression begins even when IRS desquamation is in its incipient phase. Also, K75 has a characteristic pattern of expression in telogen follicles. Conclusions: K75 expression is closely associated with the process of desquamation of the IRS. This process occurs prematurely (below the isthmus) in many follicles from patients with, but not without, CCCA. K75 expression highlights premature desquamation of the IRS in CCCA, but may not be directly involved in disease pathogenesis. Sperling LC, Hussey S, Sorrells T, Wang J‐A, Darling T. Cytokeratin 75 expression in central, centrifugal, cicatricial alopecia – new observations in normal and diseased hair follicles.     

Frontal pattern hair loss among Chinese women is frequently associated with ponytail hairstyle

Female alopecia patients in China often present with a frontal pattern hair loss. This feature is seen less frequently in western women. Women in China frequently style their hair in a ponytail. Thus, we hypothesized that the high prevalence of frontal pattern hair loss in Chinese women is due to traction alopecia. To better understand this, we studied 43 women presenting frontal alopecia at a hair clinic. Among the women presenting frontal alopecia, 79% reported styling their hair in a ponytail four or more days per week for an average period of 10 years. To our knowledge, this is the first study to elucidate the cause of frequent frontal alopecia in Chinese women. Although cultural style changes are difficult to change, we believe that awareness of the medical implications of frequent ponytail styling will reduce the rate of frontal alopecia among Chinese women.     

Permanent alopecia after busulfan chemotherapy

Systemic chemotherapy is a well known cause of reversible hair loss. Busulfan chemotherapy, however, is responsible for a permanent alopecia that usually occurs in bone marrow transplant patients. We report two patients with permanent alopecia due to busulfan chemotherapy. Both patients had a diffuse alopecia characterized by greatly reduced hair density with short, thin hair. The pathology showed reduced follicular density in the absence of fibrosis, suggesting that alopecia may result either from hair follicle stem cell destruction or from acute damage to the keratinocytes of the lower portion of some follicles.