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.     

Update on cicatricial alopecia

Cicatricial alopecia is an enigmatic group of hair disorders linked by the potential permanent loss of scalp hair follicles in involved areas. Progress in our understanding and treatment of these disorders has been stymied by the lack of clear diagnostic criteria for the current terms used to describe the various hair loss entities. Since all of these conditions evolve as the hair is destroyed or replaced, diagnosis is further made difficult by a lack of clinical and pathologic "snapshots" over the evolution of each disorder. Without some acceptance of general clinical and histological presentations in the early, mid and late stage of these disorders, one cannot begin to explore ways to make the diagnosis at a very early stage before significant follicular destraction has occurred (making the clinical diagnosis obvious) and when the damage is potentially repairable or progression preventable.     

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.     

Tissue expansion in cicatricial alopecia

A surgical approach for the treatment of cicatricial alopecia may be considered when the causal component has resolved and hair replacement is desired. Traditional procedures have been limited to scalp reduction and hair transplantation. The advent of expandable silicone implants under adjacent normal hair-bearing skin for the creation of scalp flaps offers another approach for scalp reconstruction. Tissue-expansion augmented scalp reduction of cicatricial alopecia is a surgical procedure for some patients who might not otherwise be candidates for surgical hair replacement.     

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.     

A proposed mechanism for central centrifugal cicatricial alopecia

Central centrifugal cicatricial alopecia (CCCA) has an unknown mechanism. Analyzing other scarring diseases (lichen planopilaris, fibrotic kidney disease and scleroderma) may help to clarify the mechanism of scarring in CCCA. These diseases were chosen for comparison due to either their location of disease (skin or scalp specifically), or prominence in patients of African descent. Genetics, possible triggers, an autoimmune lymphocytic response, and epithelial to mesenchymal transition are potentially involved. Possible common pathways in scarring diseases and a better understanding of the CCCA mechanism will lead to further research into the pathogenesis and potential treatments of CCCA.     

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.     

The medical treatment of cicatricial alopecia

The best outcome of current treatments of cicatricial alopecia is induction of a clinical remission with arrest of symptoms and signs, but the progression of hair loss may continue insidiously. Current treatments do not arrest the underlying disease process. A scalp biopsy is the first step in management. Selection of treatment described herein is guided by the histopathologic findings, including the type, location and extent of the predominant cellular inflammatory infiltrate, and clinical disease activity. Cicatricial alopecias with predominantly lymphocytic infiltrates are treated with immunomodulating agents, and those with predominantly neutrophilic infiltrates are treated with antimicrobial agents. Treatment selection may be challenging and requires flexibility, as histopathologic features frequently overlap, are not clear cut, or change over time. In the future, cellular and molecular biology studies will hopefully identify unique markers for the clinically distinct cicatricial alopecias and lead to better treatments and a cure.