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central centrifugal cicatricial alopecia

Thursday 3 November 2022

hot comb alopecia ; follicular degeneration syndrome

PO

Definition : Central Centrifugal Cicatricial Alopecia (CCCA) is a type of central hair loss found almost exclusively in black women.

People with CCCA often have burning, itching, tenderness, or tiny bumps on the scalp.

Variant PADI3 in CCCA.

Central centrifugal cicatricial alopecia (CCCA) is a progressive form of scarring alopecia that is most commonly seen in young to middle-aged women of African descent, but has been reported also in Black men.

The aetiology is unknown, but patients often report a history of traumatic hairstyling involving a combination of hair straighteners and perms, oils, heat, chemicals and traction.

No history of trauma associated with hairstyling is present in men.

The possibility of genetic and autoimmune involvement in the pathogenesis has been postulated.

Clinically, CCCA typically starts at the vertex or crown of the scalp, and spreads centrifugally. it progresses slowly and in time burns itself out.

In the early stages, it may show associated features of folliculitis decalvans, with pustules, crusting and erythema with bacterial superinfection.

Synopsis

- Premature desquamation of the inner root sheath in an uninflamed hair follicle

- scar tissue replacing hair follicle
- cicatricial alopecia / scarring alopecia
- near-total loss of follicular units and sebaceous glands with replacement by follicular scars.
- residual hair follicles with perifollicular fibrosis and eccentric thinning of the follicular epithelium
- premature desquamation of the inner root sheath i
- Subcutaneous tissue: numerous end-stage fibrous tracts

- typical scarring alopecia

  • perifollicular concentric fibrosis,
  • mild perifollicular and perivascular lymphoid cell infiltrate,
  • destruction of the follicular epithelium,
  • naked hair shafts in giant cells,
  • follicular dropout

- The most distinctive finding is found below the isthmus :

  • premature desquamation of the inner root sheath
  • eccentric thinning of the follicular epithelium

Whereas desquamation of the inner root sheath is a feature normally observed at the isthmus, its presence below this level indicates pathology.

Premature desquamation of the inner root sheath can be found in other inflammatory conditions of the scalp, including LPP. However, in the latter instance, the follicles are damaged by the inflammatory cell infiltrate, and represent an ‘end-stage’ follicle.

In CCCA premature desquamation of the inner root sheath may be observed also in otherwise normal, unaffected hair follicles, suggesting that it is a characteristic feature of this entity.

In vertical sections, thickened dermal elastic fibres in a hyalinized dermis have been reported.

Numerous end-stage fibrous tracts replaced by amorphous connective tissue, consistent with follicular scars, are seen in the subcutaneous tissue.

See also

- alopecia

  • diagnosis of alopecia