Central Centrifugal Cicatricial Alopecia (CCCA)

Abstract: 46 year old Ghanaian woman with scarring alopecia

HPI: The patient is a 46 yo woman from Ghana with 3 - 4 mo history of progressive alopecia. She has lived in the U.S. for ten years, takes no medications p.o. and has used hot combs only infrequently in the past.

The Examination shows patchy areas of complete hair loss on frontal, parietal and vertex areas of the scalp.
Clinical Photos:


Pathology: (Photomicrographs courtesy of Marjan Mirzabeigi, M.D. Department of Dermatopathology, Boston University School of Medicine.)









These show: Marked decrease in the number of follicular units which have been replaced with extensive fibrosis.

Diagnosis: Central Centrifugal Cicatricial Alopecia (CCCA)

Discussion: Dr. Lynn Goldberg, Boston University Department of Dermatology: "The patchy alopecia in the vertex is consistent with CCCA. If the frontal loss is contiguous it could be CCCA, although these patients often have coexistent traction. My first line of therapy is a topical steroid. Most patients will experience stabilization. I reserve ILK and doxycycline for those patients with persistent symptoms and loss, or for those patients who also have pustules, which, in my experience, is infrequent. Some physicians will start with 6 months of topical and intralesional steroids and doxy. There are no controlled trials!

Reference:
Gathers RC, Lim HW. Central centrifugal cicatricial alopecia: past, present, and future. J Am Acad Dermatol. 2009 Apr;60(4):660-8.
Abstract: Clinical scarring alopecia in African American women has been recognized for years. The classification of this unique form of alopecia dates back to Lopresti, who first described the entity called "hot comb alopecia." More recently, the term "central centrifugal cicatricial alopecia" has been adopted to describe a progressive vertex-centered alopecia most common in women of African descent. While this form of hair loss is widely recognized, and may even be on the rise, the causes of central centrifugal cicatricial alopecia are a constant source of debate and remain to be elucidated. This review outlines the descriptive evolution of central centrifugal cicatricial alopecia and the historical controversies ascribed to its pathoetiology; it also examines African hair structure and discusses how hair structure along with common physical and chemical implements utilized by individuals with African hair type may play a causal role in the development of central centrifugal cicatricial alopecia.

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Alopecia Universalis

HPI: The patient is a 77-year-old woman who was seen for alopecia, which has been present for about eight months now. This followed chemotherapy for nonhodgkin’s lymphoma. She has a history of alopecia areata decades ago which resolved on its own.

O/E: The examination shows that this patient has alopecia universalis. She has a few eyelashes but no eyebrows, no body hair, no scalp hair.

DX: Alopecia universalis following chemotherapy. This is unusual. There is one report of alopecia universalis following treatment for hepatitis C with ribavirin and interferon.

PLAN: I am going to get a list from her of the medications she was treated with for NHL and see if there are any reports on this. I will also run this be some colleagues.

Question: Has anyone seen a similar patient?

References:

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Traction Alopecia

Abstract: 15 yo girl with one year history of alopecia
HPI: This 15 yo African-American girl has noted progressive alopecia for the past year or so. Earlier in her life her hair was in corn-rows for one to two years. She has used "relaxers" for many years but stopped ~ a year ago. Her hair was pulled back for many years. Her mother has been applying "fish oil" to the area which they think may be helping.
O/E: There is marked thinning of the hair at the temporal and occipital areas. Much less involvement on frontal and parietal areas. No inflammation, scaling or scarring is appreciated.
Photos:







Diagnosis: This is most likely " Marginal Traction Alopecia"
Questions: What would you offer this young woman as for treatment. I told her to leave her hair natural, avoid relaxers or any tension on hair.
References:
1. eMedicine.com has a good chapter on Traction Alopecia: Here is an excerpt: "Traction alopecia is a common cause of hair loss due to pulling forces exerted on the scalp hair. This excessive tension leads to breakage in the outermost hairs. This condition is seen in children and adults, but it most commonly affects African American women. The 2 types of traction alopecia are marginal and nonmarginal. Unlike trichotillomania, a psychiatric disorder of compulsive hair pulling that leads to patchy hair loss, traction alopecia is unintentionally induced by various hairstyling practices (eg, use of braids, hair rollers, weaves, twists, locks, or "cornrows"). In the initial stages, this hair loss is reversible. With prolonged traction, alopecia can be permanent. Physicians, especially dermatologists, must recognize this condition early to prevent irreversible hair loss."

2. I would recommend renting Chris Rock's documentary "Good Hair" when it is available.

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Alopecia in a Child

This 11 yo boy has had this alopecic area since infancy. He has been with adoptive parents since he was a baby and his mother says this has been here since coming to live with her. At first, I thought this was a nevus sebaceous, but the scalp looks normal here with none of the raised "pebbly" surface seen in this disorders in older children.



My working diagnosis here is "Congenital Triangular Alopecia."

References: (supplied by Brian Maurer)
1. Elmer KB, George RM. Congenital triangular alopecia: a case report and review. Cutis. 2002 Apr;69(4):255-6.
Congenital triangular alopecia is a nonscarring loss of hair mass on the scalp's temporal regions. The area of hair diminution commonly is described as triangular or lancet shaped. Although previously considered congenital, this condition usually is noticed after 2 years of age and, more recently, is thought to be acquired. We propose that this entity be renamed triangular alopecia. Because this condition involves normal rather than inflamed skin, it does not respond to topical or intralesional steroids. It is important to make the correct diagnosis to avoid unnecessary and potentially harmful interventions. We present the case of a 10-year-old boy with triangular alopecia.

2. Congenital Triangular Alopecia occurring in sisters. Full Text. Original in Portugese

3. García-Hernández MJ, Rodríguez-Pichardo A, Camacho F. Congenital triangular alopecia (Brauer nevus). Pediatr Dermatol. 1995 Dec;12(4):301-3.Department of Medical-Surgical Dermatology and Venereology, Virgen Macarena University Hospital, Seville, Spain.
Abstract: Congenital triangular alopecia is manifested at 3 to 5 years of age by unilateral or, less frequently, bilateral patches of alopecia in the frontotemporal region. At this age the differential diagnosis is important, particularly as regards alopecia areata. Only about 47 cases have been reported, probably because the lesion is benign and nonprogressive. In 6200 patients seen in index visits, we found 7 with triangular alopecia, a frequency of 0.11%. We believe that males do not require treatment because of the later development of androgenic alopecia, but in women, surgical treatment is successful.2. Tosti A. Congenital triangular alopecia. Report of fourteen cases.

4. Tosti A. Congenital triangular alopecia. Report of fourteen cases. J Am Acad Dermatol. 1987 May;16(5 Pt 1):991-3.
Abstract: Fourteen patients affected by congenital triangular alopecia are presented. The clinical and histologic features of this condition are discussed. I suggest that the condition is considerably more common than hitherto has been thought.

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