Cases Of Frontal Fibrosing Alopecia Research Articles

First Italian case of frontal fibrosing alopecia in a male.

First Italian case of frontal fibrosing alopecia in a male.

Frontal Fibrosing Alopecia - a review and a practical guide for clinicians.

Despite a significant increase in reported cases of frontal fibrosing alopecia (FFA) in literature, discussion about the possible role of environmental factors, instruction for diagnosis and guideline for treatment, are limited. The review aims to provide a detailed synthesis of this condition that could be used by clinicians in their practise. Whether single-centre or multi-centre, studies of more than 60 cases less than 5 years old were mainly taken into consideration. Results obtained were that FFA affects mainly postmenopausal Caucasian women; the most common comorbidities are hyperlipidaemia, arterial hypertension, osteoporosis, hypothyroidism, depression, alongside dermatological disorders such as atopic dermatitis, rosacea, seborrheic dermatitis and androgenetic alopecia. Autoimmune, genetic, hormonal (e.g. estrogen deficiency, pregnancy, lactation, HRT and raloxifene) and environmental (e.g. daily use of facial sunscreens and less frequent use of hair dyes and shampoo) hypotheses were proposed for pathogenesis, as well as association with various predisposing factors (patient's health-social profile, disease's history and comorbidities). Clinical presentation of FFA can be divided into 3 specific patterns, each with a different prognosis. Diagnosis is usually made clinically with the use of trichoscopy; however, scalp biopsy remains the gold standard. The condition is regarded as a variant of lichen planopilaris (LPP) due to the similarity of the prominent histopathological findings, but the clinical image is distinct and therapeutic options vary. 5α-reductase inhibitors, intralesional steroids, and hydroxychloroquine provide the highest level of evidence for the treatment of FFA. The conclusion is that a better understanding of the disease is crucial for proper disease management.

The “spade sign” as a pathognomonic histopathologic feature of acne keloidalis: Analysis of 33 cases of cicatricial alopecia

To the Editor: Historically, the specific diagnosis of cicatricial alopecia using histology alone has remained elusive or impossible.1Mirmirani P. Willey A. Headington J.T. Stenn K. McCalmont T.H. Price V.H. Primary cicatricial alopecia: histopathologic findings do not distinguish clinical variants.J Am Acad Dermatol. 2005; 52: 637-643Abstract Full Text Full Text PDF PubMed Scopus (115) Google Scholar Acne keloidalis (AK) is a form of primary cicatricial alopecia, characterized by a mixed (lymphocytic and neutrophilic) infiltrate centered in the follicular isthmus. With time, there is thinning of the isthmic follicular epithelium and, eventually, clinically keloidal scarring associated with a foreign body granulomatous reaction, that is secondary to follicular destruction. Patients with AK exhibit features overlapping with other forms of alopecia.2Olsen E.A. Bergfeld W.F. Cotsarelis G. et al.Summary of North American Hair Research Society (NAHRS)-sponsored workshop on cicatricial alopecia, Duke University Medical Center, February 10 and 11, 2001.J Am Acad Dermatol. 2003; 48: 103-110Abstract Full Text Full Text PDF PubMed Scopus (291) Google Scholar In their recent AK series, Cheng et al3Cheng A.Y. Lee C.N. Hsieh F.N. et al.“Spade sign” and inflammation/fibrosis limited to the upper and mid-dermis as the pathognomonic features of acne keloidalis.J Dermatol. 2020; 47: 41-46Crossref PubMed Scopus (3) Google Scholar proposed that the spade sign (SS) is a pathognomonic histopathologic feature. They defined the SS as a spade-shaped space (resembling the spade symbol in playing cards) created by the dilated and thinned follicular epithelium in the lower isthmus. They also coined the term “balloon sign” (BS) as a variant, identical to SS, except for its round shape. The required presence of trichilemmal keratinization is characterized by the presence of isthmic follicular keratinocytes with pale eosinophilic cytoplasm and by abrupt keratinization, forming compact eosinophilic orthokeratin. To evaluate the sensitivity and specificity of the SS in a clinically relevant differential diagnosis, we performed a case control study to search for the SS in a spectrum of clinically confirmed cases of cicatricial alopecia from the teaching files of the senior author (Dr Fung). The vertical sections of hematoxylin-eosin–stained specimens were analyzed for the presence of the SS or BS. Thirty-three specimens from different patients were assessed. The diagnoses included AK (9 patients), folliculitis decalvans (6 patients), central centrifugal cicatricial alopecia (4 patients), dissecting cellulitis (4 patients), lichen planopilaris (6 patients), frontal fibrosing alopecia (2 patients), traction alopecia (1 patient), and discoid lupus (1 patient). The SS was found in 3 (3/9, 33%) AK specimens (Fig 1). Two specimens had the SS and BS in different affected follicles, whereas the third case had only the SS. The SS or BS was not present in the other cases. Upon comparing AK with all other forms of cicatricial alopecia, the sensitivity and specificity of the SS or BS for AK was found to be 33% and 100%, respectively. A single structure in 1 case of frontal fibrosing alopecia was classified as “pseudo-SS.” Although the follicular epithelium was dilated and thinned, it was located superficially and contained basket-weave orthokeratin. This superficial milia-type plugging, containing basophilic orthokeratin, may be seen in patients with frontal fibrosing alopecia and should not be confused with the SS (Fig 2).Fig 2Pseudospade sign or pseudo-balloon sign in a case of frontal fibrosing alopecia. The more superficial location of this structure is associated with epidermal keratinization in its superior portion, including a granular layer and loose, basophilic orthokeratin content, as is characteristic of comedones, milia, and epidermal inclusion cysts. (Original magnification: ×100).View Large Image Figure ViewerDownload Hi-res image Download (PPT) Our study suggests that the SS or BS is a highly specific feature of AK. Cheng et al3Cheng A.Y. Lee C.N. Hsieh F.N. et al.“Spade sign” and inflammation/fibrosis limited to the upper and mid-dermis as the pathognomonic features of acne keloidalis.J Dermatol. 2020; 47: 41-46Crossref PubMed Scopus (3) Google Scholar also identified the SS or BS in exactly 33% (5/15) of their cases. The low sensitivity of the SS may reflect the fact that the SS or BS may only be appreciated during subacute AK, prior to follicular destruction. Although we have tended to regard AK and folliculitis decalvans as very similar histologically, we did not encounter SS in our folliculitis decalvans cases. Whether this represents an artifact of insufficient numbers of cases or a true difference in diagnostic specificity warrants further study. Our source of cases may represent a limitation because the teaching cases might have been biased toward more representative features than randomly selected cases. In summary, our findings support the fact that the SS is a highly specific or pathognomonic histopathologic feature of AK. Although the absence of the SS does not argue against AK, the presence of the SS supports its diagnosis. None disclosed. The authors thank Dr María Rashidi Springall, PhD, for assistance with the statistical analysis.

A large familial cluster and sporadic cases of frontal fibrosing alopecia in Brazil reinforce known human leucocyte antigen (HLA) associations and indicate new HLA susceptibility haplotypes.

Frontal fibrosing alopecia (FFA) is a lymphocytic scarring alopecia whose worldwide incidence is rising. Environmental triggers combined with genetic predisposition represent one of the current hypotheses in FFA aetiology. Familial clusters are opportunities to investigate the genetic basis of diseases. Assess human leucocyte antigen (HLA) genetic variability in a Brazilian sample of a large familial cluster (six sisters and one daughter) with FFA, unnafected familiar members and sporadic cases of FFA. We addressed the HLA-A, HLA-B, HLA-C, HLA-G and HLA-E genetic variability in this family and in seven sporadic FFA cases, comparing allele frequencies with those reported for the São Paulo State from Brazil. Two susceptibility haplotypes, C*17:01:01:02/B*42:01:01:01 and C*07:02:01:03/B*07:02:01:01, were identified among familial cases and also in sporadic cases. The first haplotype is rare among Brazilians, and it was not previously reported as being associated with FFA. Both alleles were found in some different unaffected familiars, what emphasizes the role of environmental triggers in disease development. HLA-A, HLA-G and HLA-E genes were not associated to familiar nor FFA sporadic cases. The identification of susceptibility haplotypes in FFA reinforces the genetic predisposition to the disease.

First Italian case of frontal fibrosing alopecia in a male

First Italian case of frontal fibrosing alopecia in a male

Follicular Unit Extraction (FUE) Hair Transplantation in Combination with Platelet Rich Plasma for the Treatment of Scarring Alopecia: A Case Series

Objective: To study the effect of addition of platelet rich plasma (PRP) to the hair transplantation for scarring alopecia. Methods: An evaluation of the patients with detailed history, clinical examination and laboratory investigations was done. Routine baseline investigations and skin biopsy were done in order to rule out any activity of the underlying disease process. Freshly prepared Platelet rich plasma (PRP) using YCELL BIO kit was injected in freshly done slits over recipient area. Results: A total of 4 cases were studied- A 26 years old male with cleft lip surgical scar with absent hair growth on the middle third of the upper lip, a 22 year old male with a 5 year old post traumatic depressed scar over the junction of medial and lateral third of the right eyebrow, a 17 year old boy with a post burn scar over his left upper lip and adjoining lower cheek area and a 55 years old post menopausal female, an already diagnosed and treated case of frontal fibrosing alopecia (FFA). Hair transplantation was performed with follicular unit extraction (FUE). Freshly prepared PRP was injected intradermally after creating the slits. In all the cases, there was a good survival rate of the healthy hair follicles, accompanied by improvement in the texture of the surrounding skin. Conclusions: Platelet rich plasma, being a cocktail of growth factors, can bring vitality to the tissue and improve its vascularity, thus making it more receptive to the transplant. Additionally, with the improved vascularity of the recipient bed and activated fibroblasts, there is accelerated hair growth and better cosmetic outcomes of the scar.

Frontal fibrosing alopecia: two case reports

Two cases of frontal fibrosing alopecia (FFA) were reported. Case 1, a 44-year-old woman, presented with progressive recession of the frontotemporal hairline for 4 years and multiple skin-colored facial papules for 2 years. Skin examination showed that the frontotemporal hairline was receded, the local skin was smooth and thin, and a few remaining fine hairs could be seen. Additionally, eyebrows, axillary and pubic hairs were partly shed, and plenty of millet-sized skin-colored papules were diffusedly distributed on the frontotemporal region and bilateral mandibular angles. Dermatoscopy showed reduced hair follicular openings, different hair shaft diameters, cicatricial white patches and perifollicular erythema. Case 2, a 55-year-old woman, presented with progressive frontotemporal hair loss for 2 years. Skin examination revealed the recession of bilateral frontotemporal hairline and partial loss of eyebrows, axillary and pubic hairs. Histopathological examinations of the 2 patients both revealed perifollicular infiltration of lymphocytes, liquefaction degeneration of basal cells and perifollicular lamellar fibrosis. Clinical manifestations and histopathological features of the 2 patients both confirmed the diagnosis of FFA. Key words: Alopecia; Frontal fibrosing alopecia

Hair’s the Question: Frontal Fibrosing Alopecia (FFA)

1. Sara Wasserbauer, MD, FISHRS (drwasserbauer{at}californiahairsurgeon.com) 1. Walnut Creek, California, USA ![][1] With the increase in women seeking surgical hair restoration, more cases of frontal fibrosing alopecia (FFA) are showing up in our offices. FFA can be

‘A detective look’ at hair biopsies from African-American patients

A patient's ethnicity can be an important clue in the diagnosis of scarring alopecia as some disorders such as traction alopecia (TA) and central centrifugal cicatricial alopecia (CCCA) are more prevalent in or exclusive to African-Americans. To perform a retrospective review of 60 scalp biopsies from African-American patients including 25 cases of CCCA, 22 cases of TA, five cases of frontal fibrosing alopecia, three cases of discoid lupus erythematosus, three cases of hair breakage and two cases of alopecia areata. Serial horizontal and vertical sections were examined. Features characteristic of the African-American scalp include: golf club-shaped bulb, elliptical shape of the hair shaft, asymmetrical outer root sheath and paired grouping of hair follicles. Clues to the diagnosis of CCCA include: premature desquamation of the inner root sheath, goggles and naked hair shafts in fibrous streamers. Diagnosis of TA is suggested by preserved sebaceous glands along with follicular miniaturization and drop-out. The clues reported here aim to help the dermatopathologists to: recognize at a glance that they are dealing with a scalp biopsy from an African-American patient; make the most probable diagnosis by connecting the clues (even if only vertical sections are present); and understand the morphological basis for the susceptibility of the African hair to damage.

Frontal fibrosing alopecia associated with generalized hair loss

We present six cases of frontal fibrosing alopecia, in which generalized hair loss is a feature. Although this variant of lichen planopilaris has been reported clinically in a number of patients, there is very little histological evidence that the condition exists in peripheral sites. We believe this pattern of involvement may be more common than is reported, and have provided histological evidence of lichen planopilaris being present at sites beyond the scalp and eyebrows.

Alopecia frontal fibrosante: relato de seis casos

Alopecia frontal fibrosante é forma progressiva de alopecia cicatricial. Os casos iniciais foram relatados a partir 1994, na Austrália, em pacientes do sexo feminino pós-menopausa. Desde então inúmeros casos foram descritos na literatura sugerindo que ela é mais prevalente do que inicialmente se supunha. Seu curso progressivo se assemelha ao da alopecia androgenética; histologicamente, entretanto, o infiltrado liquenóide é evidente. O artigo relata seis casos brasileiros e discute a alopecia frontal fibrosante dentro do grupo das alopecias cicatriciais, como variante do líquen plano pilar.

Frontal fibrosing alopecia: a survey in 16 patients

Postmenopausal frontal fibrosing alopecia (PFFA) was described by Kossard et al. as a progressive recession of the frontal hairline affecting particularly postmenopausal women. Further cases of PFFA have been reported to date, all of them considering it as a variant of lichen planopilaris on the basis of its clinical, histological and immunohistochemical features. To describe clinical features, and response to treatment of 16 cases of frontal fibrosing alopecia diagnosed at our department in the last 6 years. In addition to clinical data, biopsies and laboratory tests (antinuclear antibodies, sex hormones, thyroid hormones) were performed in order to rule out other causes of scarring alopecia. Patients were treated with intralesional corticosteroids, finasteride, and minoxidil, depending on the stage of the disease and association to androgenetic alopecia. All patients presented progressive alopecia localized to the frontal and temporal hairlines. Eight patients (50%) had loss of eyebrows, and six patients (37.5%) had axillar alopecia. Ages ranged from 45 to 79. Three of these women were premenopausal. Androgenetic alopecia was evident in seven patients (43.8%). All patients biopsied showed perifollicular lymphocitic infiltrate with lamelar fibrosis limited to the upper portions of the follicle. The progression of the condition stopped in most patients after a variable period on treatment. When treatment was abandoned the alopecia progressed to 'clown alopecia' appearance. Cases of Kossard's type scarring alopecia affecting premenopausal women made us consider that this condition is not exclusive of postmenopausal women. Differential diagnosis should take into account conditions like female androgenetic alopecia, fibrosing alopecia in a pattern distribution, alopecia areata, and chronic lupus erythematosus. Except for the pattern of alopecia, lichen planopilaris and frontal fibrosing alopecia are indistinguishable, thus the latter is included as a variant of lichen planopilaris. Although the disease tends to spontaneous stabilization, intralesional and topical corticosteroids, and anti-androgens may stop the progression of the disease and improve the female androgenetic alopecia that usually is associated to FFA.