Hair and systemic disease.

Hair loss (alopecia) occurs as a manifestation of numerous systemic diseases, but usually can be categorized into one of five general groups: telogen effluvium, anagen arrest, follicular destruction, hair miniaturization, and hair shaft defects. An excess of hair also can be evidence of internal disease, and there are two general categories of increased hair density: hypertrichosis and hirsutism. The basic categories of hair disease and the systemic conditions associated with them are discussed. The history, physical examination, and histopathologic data usually are sufficient to categorize the form of hair disorder and may provide a clue to the nature of the underlying systemic disease.

Scarring alopecia and the dermatopathologist.

BACKGROUND: The evaluation of patients with cicatricial alopecia is particularly challenging, and dermatopathologists receive little training in the interpretation of scalp biopsy specimens. Accurate interpretation of specimens from patients with hair disease requires both qualitative (morphology of follicles, inflammation, fibrosis, etc.) and quantitative (size, number, follicular phase) information. Much of this data can only be obtained from transverse sections. In most cases, good clinical/pathologic correlation is required, and so clinicians should be expected to provide demographic information as well as a brief description of the pattern of hair loss and a clinical differential diagnosis. RESULTS: The criteria used to classify the various forms of cicatricial alopecia are relatively imprecise, and so classification is controversial and in a state of evolution. There are five fairly distinctive forms of cicatricial alopecia: 1) chronic, cutaneous lupus erythematosus (discoid LE); 2) lichen planopilaris; 3) dissecting cellulitis (perifolliculitis abscedens et suffodiens); 4) acne keloidalis; and 5) central, centrifugal scarring alopecia (follicular degeneration syndrome, folliculitis decalvans, pseudopelade). Not all patients with cicatricial alopecia can be confidently assigned to one of these five entities, and "cicatricial alopecia, unclassified" would be an appropriate label for such cases. CONCLUSION: The histologic features of five forms of cicatricial alopecia are reviewed. Dermatopathologists can utilize a "checklist" to catalog the diagnostic features of scalp biopsy specimens. In many, but not all, cases the information thus acquired will "match" the clinical and histologic characteristics of a form of cicatricial alopecia. However, because of histologic and clinical overlap between the forms of cicatricial alopecia, a definitive diagnosis cannot always be rendered.

Acne keloidalis is a form of primary scarring alopecia.

OBJECTIVE: To better define the pathogenesis of acne keloidalis (AK). DESIGN: Prospective, blinded study of histologic material collected from 10 patients with clinically typical AK. SETTING: Outpatient dermatology clinic of a military tertiary care medical center. PATIENTS: Ten male volunteers 18 years or older with early AK lesions (1- to 4-mm firm papules on the lower occipital/nuchal region). DATA SOURCE: Biopsy specimens from small, early lesions and from clinically uninvolved skin, studied histologically with transverse sectioning. INTERVENTION: Three separate 4-mm punch biopsy specimens of the scalp (lesional, perilesional, and "normal" scalp) were obtained from each volunteer. The specimens were processed using transverse sectioning. MAIN OUTCOME MEASURES: The primary variables for data analysis were the presence or absence of the following histologic features: premature loss of the inner root sheath; eccentric placement of shaft, with thinning of the outer root sheath; lamellar fibroplasia surrounding the follicle; loss of sebaceous glands; evidence of follicular destruction or scarring; inflammation; and intrafollicular or perifollicular microorganisms. The number and type of hairs were also recorded. RESULTS: The most common findings in the 19 histologically abnormal specimens were perifollicular, chronic (lymphocytic and plasmacytic) inflammation, most intense at the level of the isthmus and lower infundibulum; lamellar fibroplasia, most marked at the level of the isthmus; complete disappearance of sebaceous glands, associated with inflamed or destroyed follicles; thinning of the follicular epithelium, most marked at the level of the isthmus; and total epithelial destruction (superficial and deep), with residual "naked" hair fragments. Even some "normal" specimens contained true follicular scars, demonstrating that normal-appearing scalp skin had previously been affected by the disease. CONCLUSIONS: Acne keloidalis is a primary form of scarring alopecia, and many of the histologic findings closely resemble those found in certain other forms of cicatricial alopecia. Extensive subclinical disease may be present in patients with AK and can account for some of the permanent hair loss. Overgrowth of microorganisms does not appear to play an important role in the pathogenesis of the disease. There is no etiologic relationship between AK and pseudofolliculitis barbae. Therapies found to be useful in other forms of inflammatory scarring alopecia are useful in the treatment of early AK.

Hair density in African Americans.

BACKGROUND: The meager data on normal hair density in humans have been gathered from a predominantly white population. Examination of scalp biopsy specimens from African Americans suggests that hair density in this group may be lower than in whites. This study was performed to quantify any differences between white and African American patients. DESIGN: A retrospective case series of subjects who had undergone a biopsy of clinically healthy scalp skin. The 4-mm punch biopsy specimens were sectioned, and all follicles contained within the specimens were counted at various levels (suprabulbar, isthmus, and infundibulum) to arrive at the number and type of hairs present. SETTING: Outpatient clinic in a tertiary care medical center. PATIENTS: A consecutive sample of 22 African American and 12 white patients with clinically healthy scalp skin specimens that were studied and compared with previously reported data. MAIN OUTCOME MEASURES: Patients' age and total number of follicles, terminal follicles, vellus follicles, terminal anagen hairs, and terminal telogen hairs. RESULTS: Total hair density (number of follicles per 4-mm punch biopsy specimen) and total number of terminal follicles and terminal anagen hairs were significantly lower in African Americans (P<.001) than in whites and in a previously reported, predominantly white, population. CONCLUSIONS: Hair density in African Americans is significantly lower than that in whites, which must be taken into consideration when evaluating a biopsy specimen from an African American patient. Data previously collected from white patients may not provide adequate guidance when evaluating scalp biopsy specimens from African Americans and could lead to an incorrect diagnosis.

Temporal triangular alopecia acquired in adulthood.

Temporal triangular alopecia is a relatively common, nonscarring form of alopecia. Sometimes congenital, the vast majority of lesions appear during the first 6 years of life and remain stable thereafter. We report a case of temporal triangular alopecia arising during adulthood.

Hair diseases.

A few common causes of hair loss account for the vast majority of cases. A well-directed history and simple physical examination is often sufficient to make a diagnosis. Laboratory testing is often unnecessary. A scalp biopsy can be useful, but only if processed and interpreted correctly. Androgenetic alopecia, alopecia areata, senescent alopecia, telogen effluvium, traction alopecia, trichotillomania, and cosmetic hair damage are common causes of non-scarring alopecia. Discoid lupus erythematosus, lichen planopilaris, and central, centrifugal scarring alopecia are the most common forms of scarring hair loss.

Transverse scalp sections: a proposed method for laboratory processing.

BACKGROUND: Transverse sections of human scalp biopsy specimens can provide both qualitative and quantitative information about follicular histopathology not readily available in vertically sectioned specimens. OBJECTIVE: We propose a method for making the processing and interpretation of such specimens easier. METHODS: All scalp biopsy specimens obtained during the past 18 months in our institution for the evaluation of alopecia were processed for transverse sections by means of a technique employing trisection or quadrisection (rather than the standard bisection), and maintaining all sections in the same anatomic orientation (deep to superficial) in all tissue pieces on microscopic slides. RESULTS: More than 120 transversely sectioned specimens from more than 75 patients have been processed with this technique. The typical number of slides cut per specimen decreased from between 12 and 20 to between 1 and 4, with no loss of clinical information. CONCLUSION: This technique allows transverse sections for evaluation of alopecia to be processed in a more cost-effective manner and compares favorably with previously published techniques in providing diagnostic information.

Loose anagen hair syndrome mimicking the uncombable hair syndrome.

A 7-year-old female presented with messy, difficult to manage scalp hair and mild, diffuse alopecia. Hair pull specimens, diagnostic for loose anagen hair syndrome, also showed hair shaft abnormalities described in the uncombable hair syndrome. We suggest that dysmorphic hair shafts observed on our patient account for her clinically unmanageable hair. Pertinent clinical, pathologic, and diagnostic features of both syndromes are reviewed.

Essential syphilitic alopecia revisited.

There has been a resurgence of syphilis in the past decade. Uncommonly, diffuse hair loss, termed essential alopecia, is the only sign of syphilitic infection. We describe two patients with syphilis in whom the first sign of disease was alopecia and discuss the clinical and histopathologic findings of essential syphilitic alopecia.

Phialophora verrucosa: a new cause of mycetoma.

A 29-year-old Thai woman had draining sinus tracts, tumefaction, and granules on the plantar aspect of the foot. Phialophora verrucosa was isolated from the lesion. P. verrucosa is a major agent of chromoblastomycosis, which is known to rarely cause subcutaneous phaeohyphomycosis. This dematiaceous fungus has not been previously reported to cause mycetoma. This case illustrates the clinical spectrum of disease of this fungus. The salient features of mycetoma and management options are presented.

Clinical and histologic findings in temporal triangular alopecia.

BACKGROUND: Temporal triangular alopecia (TTA; also called "congenital triangular alopecia") is a common disorder that is assumed to be congenital. Little is known about its histologic features. OBJECTIVE: Our purpose was to describe four new cases, review the literature, and present histologic features based on vertical and transverse sectioning. METHODS: The history, clinical features, and histologic findings of four patients with TTA are described and the relevant literature reviewed. RESULTS: Lesions of TTA are seldom congenital, and most are best described as lancet-shaped. The "bald spot" contains normal numbers of hairs, although virtually all are vellus or indeterminate follicles. CONCLUSION: Most cases of TTA appear to develop during the first few years of life, and the designation "congenital" is a misnomer. The appearance of alopecia can be best explained as a focal zone of hair miniaturization leading to vellus hair formation.

Follicular degeneration syndrome in men.

BACKGROUND AND DESIGN: Follicular degeneration syndrome (FDS, formerly called hot comb alopecia) has only been described in black women. The clinical and histologic features of eight black men with a scarring alopecia resembling FDS were studied. OBSERVATIONS: All eight men had evidence of scarring alopecia, which was most prominent on the crown of the scalp. None of the men were using chemical or physical modalities to straighten or style the hair. Overall, this group of men had more evidence of active inflammation than did the previously described women with FDS. The histologic features in the men were identical to those found in women with FDS. The presence of premature desquamation of the inner root sheath and migration of the hair shaft through the outer root sheath serve as histologic markers of FDS and separate it histologically from other forms of scarring alopecia. CONCLUSIONS: Follicular degeneration syndrome is a common form of scarring alopecia in black men, just as it is in black women. In men, there is no association between chemical or mechanical hair styling techniques (eg, the "hot comb") and onset, progression, or severity of disease. The histologic features of FDS in men are identical to those in women.

Bowen's disease of the nail bed and periungual area. A clinicopathologic analysis of seven cases.

BACKGROUND: This article describes the clinical and histologic features of seven cases of Bowen's disease (BD) of the nail bed, evaluates the role of human papillomavirus in the bowenoid change, and discusses optimal therapy. OBSERVATION: The patients presented with the clinical features of verruca vulgaris (n = 3), nail dystrophy and onycholysis (n = 2), paronychia (n = 1), and acral melanoma (n = 1). Histologically, the lesions demonstrated acanthosis, hyperkeratosis, and anaplasia, involving the full thickness of the epithelium. In four cases, human papillomavirus type 16 was demonstrated by in situ hybridization. Six lesions were treated by Mohs micrographic surgery, and one case was treated with topical 5% fluorouracil. In two cases, lesions recurred 1 and 2 years following surgery. In the case treated with topical 5% fluorouracil, residual BD was found 6 weeks after therapy. This case was then treated by Mohs surgery. CONCLUSIONS: Bowen's disease of the nail bed and periungual area may present clinically as various inflammatory and neoplastic conditions. An important clinical finding in differentiating BD of the nail bed from verruca is the presence of scaling and onycholysis that are out of proportion to the verrucous changes. Human papillomavirus type 16 may be etiologically related to BD of the nail bed and periungual area. Mohs micrographic surgery is recommended for adequate excision and maximal preservation of normal tissue and function.