Scarring Alopecias are divided into Primary and Secondary Cicatricial Alopecias. In the case of primary, the hair follicle is the primary target of destruction. In the case of secondary, follicular destruction is incidental to a non-follicular process such as infection; traction; tumor; burn.

The clinical hallmark of Scarring Alopecias is the loss of follicular orifices and this finding dictates a scalp biopsy. In the case of Primary Cicatricial Alopecias, although the clinical variants are distinct, histopathology cannot distinguish these clinical variants , and can only separate them into two groups:
1) Predominantly lymphocytic group – LMPCA
2) Predominantly Neturtophylic groups – NMPCA

Generally Primary Cicatricial Alopecias affect adults and are rare in children, occur worldwide, and there is seldom a family history with the exception of Central Centrifugal Cicatricial Alopecia (CCCA).

A working classification of Primary Cicatricial Alopecia is based on pathological changes and the predominant cellular infiltrate, whether Lymphocytic; Neturophylic or Mixed; or End Stage.

Lymphocytic Group – Lymphocytic Mediated Primary Cicatricial Alopecia (LMPCA)
Lichen Plano Pilaris; Frontal Fibrosing Alopecia; Central Centrifugal Cicatricial Alopecia; Pseudopelade of Brocq; Graham Little Syndrome;

Neutrophylic Group – Neutrophylic Mediated Primary Cicatricial Alopecia (NMPCA)
Folliculitis Decalvans; Tufted Folliculitis

Mixed Group:
Dissecting Cellulitis; Folliculitis Kelloidalis Nuchae

End Stage:
Non Specific and different types are indistinguishable histologically

It is essential to explain to patients that the goal of treatment is to alleviate the symptoms and signs and to retard or slow progression of the disease. Hair regrowth is not possible at this time and activity may recur after months or years.

New research suggests that in some Primary Cicatricial Alopoecias, the peri-follicular inflammation may be secondary to lipid-metabolic changes in the sebaceous gland, specifically in some patients with Lichen Plano Pilaris, there is a loss of function of the Peroxisome Proliferator Activated Receptor Gamma (PPAR-Gamma) in the Sebacious Gland, resulting in the buildup of toxic lipids and triggers inflammation and eventually in scarring and destruction of the follicle. Damage is caused to the Stem Cells. For this reason, a PPAR –Gamma agonist is listed as a new “off label” treatment option for LPP.

Hair restoration surgery is not often considered in patients who suffer from Primary Cicatricial Alopecias. Some hair restoration surgeons have reported successful hair transplants after two years of confirmed disease remission, however, several have reported flare-ups of the Scarring Alopecia at a later date with resultant loss of transplanted hair when the disease flares up again.

Hair transplants are contra-indicated in Scarring Alopecia’s when patients are symptomatic, presenting with rapid hair loss, patchy hair loss, loss of follicular markings, peri-follicular inflammation, and with itching and burning sensations of the scalp. A scalp biopsy is required to confirm that the disease has burnt out or gone into remission and only then can a hair transplant be considered. Patients who then choose to undergo hair restoration surgery are advised to also take the indicated medical treatment programs on an ongoing basis to prevent flare-up.

Sources:
– Cicatricial Alopecia, An Approach to Diagnosis and Management, by Dr’s V Price and P Mirmirani
– Lecture on Cicatricial Alopecia at ISHRS Conference, San Francisco, 2015, Dr V Price; Dr P Mirmirani; Dr P Rose
– C.A.R.F: Cicatricial Alopecia Research Foundation – www.carfintl.org

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