Primary Scarring Alopecia: Clinical-Pathological Review of 72 Cases and Review of the Literature

Abstract

Purpose of the study: To analyze the epidemiologic, demographic, clinical, and histological characteristics of primary scarring alopecia (PSA) cases diagnosed over a 7-year period at the Department of Dermatology, Hospital Clinic, Barcelona, Spain.

Procedures: Seventy-two patients diagnosed with PSA between 2006 and 2012 were included. Age, sex, ethnic group, clinical pattern, predominant histological infiltrate, final clinical diagnosis, time of onset, treatments used, and clinical evolution were evaluated and correlated.

Results: The ethnic groups were distributed as follows: 93% European-Caucasian, 5% Mestizo-American, 1% oriental, and 1% Afro-American. Most cases were females (71%), and mean age was 51 ± 6 years. The follicular pattern was the most common, and the predominant inflammatory infiltrate was lymphocytic. Lichen planopilaris and frontal fibrosing alopecia were the main diagnoses. When correlating clinical aspects and histopathology, lymphocytic PSAs had a subacute onset and resulted in a nonchanging, more stable form, while neutrophilic PSAs had a more acute onset with an evolution of acute outbreaks. PSAs in a late stage with an absent/mild infiltrate had a subclinical onset and a slowly progressive or stable evolution.

Conclusions: The PSAs are severe trichological conditions. Their high clinical and histopathological variability make them a diagnostic and therapeutic challenge.

Message of the paper: Knowing the clinical and histopathological aspects of PSAs should be of crucial importance to the dermatologist.

Keywords: Cicatricial alopecia; Lymphocytic scarring alopecia; Neutrophilic scarring alopecia; Pseudopelade; Scarring alopecia.

Fig. 1

Predominant types of inflammatory infiltrate observed in the studied cases. The lymphocytic infiltrate was most commonly observed, followed by an absent/mild infiltrate.

Fig. 2

Examples of characteristic predominant lymphocytic (a) and neutrophilic (b) infiltrates in scalp biopsies.

Fig. 3

Most frequent clinical patterns. The most commonly observed were a follicular pattern and a single large patch.

Fig. 4

Examples of the clinical patterns observed. a Follicular. b Single large patch. c Multiple patches. d Marginal. e “Footprints in the snow.” f Folliculitis decalvans. g Acne keloidalis.

Fig. 5

Type of onset observed and its correlation with the predominant inflammatory infiltrate. It is important to notice that PSAs with a lymphocytic infiltrate correlated mostly with a subacute onset, while neutrophilic PSAs with a more acute onset. PSAs with absent/mild infiltrate, which may correspond to late stages of the disease, correlated with a more subclinical form of onset.

Fig. 6

Type of clinical evolution and its correlation with the predominant inflammatory infiltrate. PSAs with a lymphocytic infiltrate more commonly correlated with a stable, nonchanging form, while neutrophilic PSAs had more acute outbreaks. PSAs with absent/mild infiltrate, most commonly corresponding to late stages, had mostly a stable clinical course.

Fig. 7

Most frequent final diagnoses. The most commonly observed were lichen planopilaris and its variant, frontal fibrosing alopecia.

Fig. 8

Treatments used more commonly in neutrophilic and lymphocytic PSAs, according to the authors' experience.