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 Table of Contents  
CASE REPORT
Year : 2018  |  Volume : 10  |  Issue : 4  |  Page : 175-179  

Linear circumscribed scleroderma-like folliculitis decalvans: Yet another face of a protean condition


1 Alvaro Alvim School Hospital, Department of Dermatology, Campos dos Goytacazes, Rio de Janeiro, Brazil, Switzerland
2 Antonio Pedro University Hospital, Center of Medical Sciences, Federal Fluminense University, Niteroi, Rio de Janeiro, Brazil
3 Kempf and Pfaltz Laboratory, Diagnostic Histopathology, Wallisellen, Zurich, Switzerland
4 Center for Dermatology and Hair Diseases Professor Trüeb, Wallisellen, Zurich, Switzerland

Date of Web Publication9-Oct-2018

Correspondence Address:
Prof. Ralph Michel Treüb
Hair Diseases, Bahnhofplatz 1A, CH-8304 Wallisellen
Switzerland
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijt.ijt_9_18

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   Abstract 


Since Quinquaud's original report of folliculitis decalvans (FD), further clinical variants have been described on the basis of common histopathological and microbiological findings. Histopathology reveals a neutrophilic primary scarring alopecia, and microbiological studies invariably reveal pathogenic strains of Staphylococcus aureus. The presence of thickening of lesional skin in FD has been previously described. We report a new presentation of FD, clinically mimicking linear circumscribed scleroderma of the scalp. Overlapping features of the scarring alopecias may blur the distinction between different conditions that ultimately share the common final pathway of replacement of follicle by fibrous tissue. Therefore, a careful patient history, clinical examination including dermoscopy, microbiological studies, and a scalp biopsy for histopathology, and immunofluorescence studies are prerequisites to an accurate diagnosis and appropriate treatment of the respective condition. The case is presented to illustrate the clinical variability in presentation of FD and to underline the necessity of performing a biopsy for an accurate diagnosis in the scarring alopecias.

Keywords: Folliculitis decalvans, folliculitis decalvans with linear arrangement, linear circumscribed scleroderma, linear circumscribed scleroderma-like folliculitis decalvans


How to cite this article:
Rezende HD, Dias MF, Kempf W, Treüb RM. Linear circumscribed scleroderma-like folliculitis decalvans: Yet another face of a protean condition. Int J Trichol 2018;10:175-9

How to cite this URL:
Rezende HD, Dias MF, Kempf W, Treüb RM. Linear circumscribed scleroderma-like folliculitis decalvans: Yet another face of a protean condition. Int J Trichol [serial online] 2018 [cited 2019 May 20];10:175-9. Available from: http://www.ijtrichology.com/text.asp?2018/10/4/175/242926




   Introduction Top


I can add colours to the chameleon, Change shapes with Proteus for advantages

–William Shakespeare

The scarring alopecias represent a group of diverse disorders with permanent destruction of the pilosebaceous unit and irreversible hair loss. Where there is no obvious physical/chemical injury or acute infectious etiology, clinical differential diagnosis of scarring alopecia is often difficult. The clinical inspection is of limited usefulness in establishing a diagnosis. Overlapping features may blur the distinction between different conditions that ultimately share the common final pathway of replacement of follicle by fibrous tissue. Therefore, a careful patient history, clinical examination including dermoscopy, microbiological studies, and a scalp biopsy for histopathology, and immunofluorescence studies are prerequisites to an accurate diagnosis and appropriate treatment of the respective condition.[1]

Linear circumscribed scleroderma of the scalp and folliculitis decalvans (FD) represent two disparate scarring alopecias that are characterized by distinctive clinical, dermoscopic, microbiological, and histopathological features. A case of linear circumscribed scleroderma-like FD is presented to illustrate the clinical variability in presentation of FD and to underline the necessity of performing a biopsy for an accurate diagnosis in the scarring alopecias.


   Case Report Top


A 39-year-old female patient was referred by her dermatologist because of a focal scarring alopecia of the vertex region with an unspecific histopathologic finding of fibrosing alopecia.

Clinical inspection revealed a 1.5 cm × 4 cm area of white porcelain-colored plaque of scarring alopecia with thickened skin in a linear pattern [Figure 1].
Figure 1: White porcelain-colored plaque of scarring alopecia with thickened skin in a linear pattern

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Dermoscopic examination of the active border showed discrete tufting, perifollicular scaling, erythema, telangiectasia, and tubular silver-white hair casts.

Histological examination revealed hyperplasia of the interfollicular epidermis with hair follicles merging to form hair tufts and a perifollicular-mixed inflammatory infiltrate with a large number of plasma cells [Figure 2]a. In the elastin stain, the dermal elastic network was effaced, consistent with diffuse dermal fibrosis [Figure 2]b.
Figure 2: (a) Histopathology (H and E): Hyperplasia of the interfollicular epidermis with hair follicles merging to form hair tufts, and a perifollicular-mixed inflammatory infiltrate with a large number of plasma cells. (b) Elastin stain: effaced dermal elastic network, consistent with diffuse dermal fibrosis

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Borrelia serology tested positive for Borrelia burgdorferi IgG with 21.0 U/mL (normal: <16 U/mL) and negative for B. burgdorferi IgM.

The patient was treated with 200 mg oral doxycycline daily and topical clobetasol propionate lotion.


   Discussion Top


FD, as originally described by Quinquaud in 1888,[2] represents a chronic and recurrent pustulofollicular scalp inflammation usually of the crown area with exudative crusted areas and grouped follicular pustules at the hair-bearing margin and centrifugal progression with central scarring. Since Quinquaud's original report, further clinical variants have been described on the basis of common histopathological and microbiological findings. Histopathology reveals a neutrophilic primary scarring alopecia, and microbiological studies invariably reveal pathogenic strains of Staphylococcus aureus although a Gram-negative bacterial folliculitis may evolve in patients who receive prolonged courses of antibiotic therapy or use antibacterial topicals that selectively inhibit Gram-positive organisms.[3]

In 1947, Laymon[4] described cicatrizing seborrheic eczema as a condition of scarring alopecia combining features of seborrheic dermatitis and FD.

In 1977, Smith and Sanderson[5] and at the same time Metz and Metz[6] reported on tufted hair folliculitis, whereby the former considered the condition to be secondary to follicular inflammation and destruction, while the latter deemed the inflammation to be secondary to preexisting nevoid hair bundles. Since the original report of tufted hair folliculitis, there has been considerable controversy whether the condition represents a distinctive entity or an unspecific finding secondary to a variety of inflammatory and scarring alopecias. Based on clinical, histopathological, and microbiological criteria, the general consensus is that the condition represents a variant of FD.[7],[8],[9] Secondary tufting of hair follicles due to other inflammatory conditions of the scalp is usually less marked with <5 hair shafts per tuft, and dermoscopic features allow for differentiation.[10] Histopathological studies reveal perifollicular inflammation around the upper portions of the follicles sparing the hair root level. Within areas of inflammation, several follicles converge toward a common follicular duct with a widely dilated opening. It is believed that the development of atrophy with loss of adnexal structures in classical FD or of hair tufts in tufting folliculitis depends on the depth and destructive potential of the inflammatory process, with sparing of the bulge area preserving an intact lower portion of hair follicles in the case of tufted hair folliculitis.[11]

Very rarely, FD can present with an extensive ulcerated appearance of the scalp that has been reported to have been misinterpreted as a laceration of the scalp at the forensic scene.[12]

Ultimately, the pseudopeladic state as originally defined by Degos[13] in 1954 represents the nonspecific end stage of a variety of at least 60 types of cicatricial alopecias, including FD. It presents with a large area of scarring with irregular borders.

The pathogenesis and clinical presentation of FD are determined by three factors: (1) the infectious pathogen, (2) the incubatory microenvironment, and (3) the host immune response.

Occasionally, FD has been linked to a specific immune deficiency,[14],[15],[16] ectodermal dysplasia,[17] or hair transplantation, either autologous[18] or synthetic,[19] impairing the immune defense mechanisms of the hair follicle. Traditionally, the medical focus has been on the condition of either the hair or the scalp. Indeed, the proximate structural arrangement of the scalp and hair leads to an interdependent relationship between the two. The role of the scalp as an incubatory environment hair has only recently received appropriate attention.[20] In fact, seborrhea, dandruff, and seborrheic dermatitis of the scalp are frequently associated with hair loss and superficial pustular folliculitis that nevertheless may eventually evolve to cicatrizing seborrheic eczema.

Chiarini et al.[21] proposed that the infection of hair follicles with S. aureus induces an intense peri- and intrafollicular migration of neutrophils, recruited by innate immunity mechanisms, involving interleukin-8 (IL-8). Furthermore, T-lymphocytes may be activated either by microbial antigens through processing by Langerhans cells or by superantigens through the Vb domain of the T-cell receptor with consecutive release of pro-inflammatory such as interferons alpha-gamma and tumor necrosis factor-alpha and profibrotic mediators such as transforming growth factor-beta, beta-fibroblast growth factor, IL-1b, and Il-4, resulting in both inflammation and fibrosis.

The observation of simultaneous occurrence of secondary cutis verticis gyrata, FD, and folliculitis keloidalis nuchae in a male patient of African origin with dreadlocks underlines a putative pathogenic role of traction or trauma and the activation of different fibroblast growth factor members.[22]

According to Pujol's[23] hypothesis, tufted hair folliculitis observed in FD and in folliculitis keloidalis nuchae is secondary to follicular damage and caused by inflammatory cytokine secretion. Accordingly, tufted hair folliculitis has been reported following scalp injury (scalp laceration).[24]

A more recent study provided evidence of the presence of bacterial biofilms in the infrainfundibular part of human scalp hair follicles in FD.[25] A biofilm is any group of microorganisms in which cells stick to each other on a surface. These adherent cells are frequently embedded within a self-produced matrix of extracellular polymeric substance. Biofilms form on living or nonliving surfaces and in the case of FD on the surfaces of the hair shaft. Bacteria living in a biofilm usually have significantly different properties from free-floating bacteria of the same species, as the dense and protected environment of the film allows them to cooperate and interact in various ways. One benefit of this environment is increased resistance to antibiotics, as the dense extracellular matrix and the outer layer of cells protect the interior of the community. Biofilms have been found to be involved in a wide variety of microbial infections in the body, and more recently, it has been noted that bacterial biofilms may impair cutaneous wound healing and reduce topical antibacterial efficiency in healing or treating infected skin wounds. The presence of a bacterial biofilm at the interface of the hair shaft may provide an explanation for the chronicity and high relapse rate of FD.

With time, FD tends to develop clinical and dermoscopic features of lichen planopilaris (LPP). More important tufting, and on histopathology, a more diffuse pattern of effaced dermal elastic fibers versus selective loss of elastic fibers at the site of selectively destroyed hair follicles,[26] in combination with a more important number of plasma cells in the inflammatory infiltrate,[27] helps distinguish late phases of FD from LPP. It can be assumed that through the destruction of hair follicles in the course of the primary infectious disease, follicular antigens are exposed and give rise to an autoimmune reaction. In fact, LPP is regarded to be a T cell-mediated autoimmune reaction in response to some antigenic challenge with apoptosis of the follicular epithelial cells. Harries et al.[28] provide the first evidence that LPP may result from an immune privilege collapse of the hair follicle's epithelial stem cell niche. Where a causal or triggering agent is identified, this is termed a lichenoid reaction rather than lichen planus; therefore, the term chronic lichenoid phase of FD is proposed for this presentation of FD.

We report yet another presentation of FD, clinically mimicking linear circumscribed scleroderma of the scalp.

Linear circumscribed scleroderma or linear morphea of the scalp is a form of localized scleroderma that characteristically affects the scalp to produce a line of ivory- or porcelain-colored thickened skin with loss of hair. The thickening and hardening of the skin and underlying subcutaneous tissue result from excessive collagen deposition. On histopathological examination, the dermal elastic network remains preserved.[26] The cause is unknown although reports of morphea coexisting with other systemic autoimmune diseases support that morphea is also a condition of autoimmune origin. It has been proposed that linear morphea follows Blaschko's lines, giving rise to the hypothesis that in patients with linear morphea, susceptible cells are present in a mosaic state, and exposure to some trigger factor, including trauma, may result in the development of this condition.[29]

Kim et al.[30] originally reported tufted hair folliculitis in a linear arrangement and again discussed Pujol's trauma hypothesis. We present the second case of histopathologically proven FD in linear arrangement, though with a linear circumscribed scleroderma-like appearance due to thickening of the epidermis. The presence of thickening of lesional skin in FD has been previously commented on. In a retrospective histological analysis of 26 patients with FD, the authors found follicular hyperkeratosis in 77%, hyperplasia of the interfollicular epidermis in 92% with a psoriasiform aspect in 88%, plasma cells in infiltrate in 92%, in 42% in large quantities, and hair tufting in 54%.[31]

We propose adding linear circumscribed scleroderma-like FD to the protean clinical manifestations of FD, and as a subset of FD with linear arrangement, as presented in [Table 1].
Table 1: Clinical variants of folliculitis decalvans

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Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Harries MJ, Trueb RM, Tosti A, Messenger AG, Chaudhry I, Whiting DA, et al. How not to get scar(r)ed: Pointers to the correct diagnosis in patients with suspected primary cicatricial alopecia. Br J Dermatol 2009;160:482-501.  Back to cited text no. 1
    
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Garcia-Bustinduy M, Lecuona M, Guimera F, Saez M, Dorta S, Sanchez R, et al. Citrobacter koseri in scalp folliculitis. Cutis 2002;69:393-4.  Back to cited text no. 3
    
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Laymon CW. The cicatricial alopecias; an historical and clinical review and an histologic investigation. J Invest Dermatol 1947;8:99-122.  Back to cited text no. 4
    
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Smith NP, Sanderson KV. Tufted hair folliculitis of the scalp. J R Soc Med 1978;71:606-8.  Back to cited text no. 5
    
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Dalziel KL, Telfer NR, Wilson CL, Dawber RP. Tufted folliculitis. A specific bacterial disease? Am J Dermatopathol 1990;12:37-41.  Back to cited text no. 7
    
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Annessi G. Tufted folliculitis of the scalp: A distinctive clinicohistological variant of folliculitis decalvans. Br J Dermatol 1998;138:799-805.  Back to cited text no. 8
    
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Powell JJ, Dawber RP, Gatter K. Folliculitis decalvans including tufted folliculitis: Clinical, histological and therapeutic findings. Br J Dermatol 1999;140:328-33.  Back to cited text no. 9
    
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Rakowska A, Slowinska M, Kowalska-Oledzka E, Warszawik O, Czuwara J, Olszewska M, et al. Trichoscopy of cicatricial alopecia. J Drugs Dermatol 2012;11:753-8.  Back to cited text no. 10
    
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Miura M, Dekio I, Yamasaki Y, Ohyama M. Sparing of the bulge area could preserve intact lower portion of hair follicles in a case of tufted folliculitis. J Eur Acad Dermatol Venereol 2009;23:87-9.  Back to cited text no. 11
    
12.
James C, Langlois NE. Folliculitis decalvans: A rare scarring alopecia misinterpreted as a laceration of the scalp at the scene. Forensic Sci Med Pathol 2013;9:594-5.  Back to cited text no. 12
    
13.
Degos R, Rabut R, Duperrat B, Leclercq R. Pseudopeladic state; comments on one hundred cases of circumscribed cicatricial alopecia, apparently primary, of pseudopelade type. Ann Dermatol Syphiligr (Paris) 1954;81:5-26.  Back to cited text no. 13
    
14.
Schwartz JR, Henry JP, Kerr KM, Flagler MJ, Page SH, Redman-Furey N, et al. Incubatory environment of the scalp impacts pre-emergent hair to affect post-emergent hair cuticle integrity. J Cosmet Dermatol 2018;17:105-11.  Back to cited text no. 14
    
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Shitara A, Igareshi R, Morohashi M. Folliculitis decalvans and cellular immunity – Two brothers with oral candidosis. Jap J Dermatol 1974;28:133.  Back to cited text no. 15
    
16.
Frazer NG, Grant PW. Folliculitis decalvans and hypocomplementemia. Br J Dermatol 1982;107 Suppl 22:88.  Back to cited text no. 16
    
17.
Wheeland RG, Thurmond RD, Gilmore WA, Blackstock R. Chronic blepharitis and pyoderma oft he scalp: An immune deficiency state in a father and son with hypercalcemia and decreased intracellular killing. Pediatr Dermatol 1983;1:134-42.  Back to cited text no. 17
    
18.
Trüeb RM, Tsambaos D, Spycher MA, Müller J, Burg G. Scarring folliculitis in the ectrodactyly-ectodermal dysplasia-clefting syndrome. Histologic, scanning electron-microscopic and biophysical studies of hair. Dermatology 1997;194:191-4.  Back to cited text no. 18
    
19.
Otberg N, Wu WY, Kang H, Martinka M, Alzolibani AA, Restrepo I, et al. Folliculitis decalvans developing 20 years after hair restoration surgery in punch grafts: Case report. Dermatol Surg 2009;35:1852-6.  Back to cited text no. 19
    
20.
Colli P, Fellas A, Trüeb RM. Staphylococcus lugdunensis and Trichophyton tonsurans infection in synthetic hair implants. Int J Trichology 2017;9:82-6.  Back to cited text no. 20
    
21.
Chiarini C, Torchia D, Bianchi B, Volpi W, Caproni M, Fabbri P, et al. Immunopathogenesis of folliculitis decalvans: Clues in early lesions. Am J Clin Pathol 2008;130:526-34.  Back to cited text no. 21
    
22.
Pereira LA, Teixeira M, Andrade J, Rochael M, Reis Gavazzoni-Dias MF. An overlap of secondary cutis verticis gyrata, folliculitis decalvans, folliculitis keloidalis nuchae an the use of dreadlocks: The role of inflammation due to traction. Skin Appendage Disord 2016;2:130-4.  Back to cited text no. 22
    
23.
Pujol RM, García-Patos V, Ravella-Mateu A, Casanova JM, de Moragas JM. Tufted hair folliculitis: A specific disease? Br J Dermatol 1994;130:259-60.  Back to cited text no. 23
    
24.
Fernandes JC, Correia TM, Azevedo F, Mesquita-Guimarães J. Tufted hair folliculitis after scalp injury. Cutis 2001;67:243-5.  Back to cited text no. 24
    
25.
Matard B, Meylheuc T, Briandet R, Casin I, Assouly P, Cavelier-balloy B, et al. First evidence of bacterial biofilms in the anaerobe part of scalp hair follicles: A pilot comparative study in folliculitis decalvans. J Eur Acad Dermatol Venereol 2013;27:853-60.  Back to cited text no. 25
    
26.
Fung MA, Sharon VR, Ratnarathorn M, Konia TH, Barr KL, Mirmirani P, et al. Elastin staining patterns in primary cicatricial alopecia. J Am Acad Dermatol 2013;69:776-82.  Back to cited text no. 26
    
27.
Otberg N, Kang H, Alzolibani AA, Shapiro J. Folliculitis decalvans. Dermatol Ther 2008;21:238-44.  Back to cited text no. 27
    
28.
Harries MJ, Meyer K, Chaudhry I, E Kloepper J, Poblet E, Griffiths CE, et al. Lichen planopilaris is characterized by immune privilege collapse of the hair follicle's epithelial stem cell niche. J Pathol 2013;231:236-47.  Back to cited text no. 28
    
29.
Weibel L, Harper JI. Linear morphoea follows Blaschko's lines. Br J Dermatol 2008;159:175-81.  Back to cited text no. 29
    
30.
Kim JH, Park HY, Lee WS. Tufted hair folliculitis with linear arrangement. Indian J Dermatol Venereol Leprol 2011;77:535.  Back to cited text no. 30
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31.
Matard B, Cavelier-Balloy B, Reygagne P. Epidermal psoriasiform hyperplasia, an unrecognized sign of folliculitis decalvans: A histological study of 26 patients. J Cutan Pathol 2017;44:352-7.  Back to cited text no. 31
    


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