An Overlap of Secondary Cutis Verticis Gyrata, Folliculitis Decalvans, Folliculitis Keloidalis Nuchae and the Use of Dreadlocks: The Role of Inflammation due to Traction

Abstract

We report a 29-year-old black male with cutis verticis gyrata, folliculitis decalvans and folliculitis keloidalis nuchae confirmed by biopsy. He had been using dreadlocks for 5 years before the appearance of the lesions. An activation of the different fibroblast growth factor members may explain the development of hyperproliferation of collagen, fibrosis and keloid lesions. We suggest a hypothesis of a common pathogenesis for the three conditions in a genetically predisposed patient. Inflammation and traction caused by the dreadlocks can act as a possible trigger factor.

Established Facts

• The growth and development of hair follicles is influenced by a number of different growth factors and cytokines, particularly members of the fibroblast growth factor family.

• Staphylococcus aureus, frequently detected in active lesions of folliculitis decalvans and early stages of folliculitis keloidalis nuchae, may act as superantigen and activate inflammation.

Novel Insights

• Simultaneous occurrence of these three diseases has not yet been reported in the literature.

• The activation of different fibroblast growth factor members in genetically predisposed patients may be a common pathological basis for the development of cutis verticis gyrata, folliculitis decalvans and folliculitis keloidalis nuchae in the same patient.

• The traction from the dreadlocks can be a trigger factor for the dermal collagen thickening present in the 3 conditions because it facilitates infection by S. aureus and sustains the inflammation.

Introduction

Cutis verticis gyrata (CVG) is a rare condition characterized by convoluted ridges and folds on the scalp, mimicking cerebral gyri, due to excessive growth of the scalp skin. CVG is classified as primary (essential and nonessential) or secondary, according to the underlying etiology. Primary essential CVG has no other associated abnormalities. Primary nonessential CVG is often associated with neuropsychiatric and ophthalmological disorders. Secondary CVG has been linked to local inflammatory skin conditions, such as eczema and psoriasis, and to systemic illnesses, such as acromegaly and pachyodermoperiostosis [1]. Folliculitis decalvans (FD) is a neutrophilic cicatricial alopecia characterized by papules, pustules and tufted hair folliculitis with a chronic, progressive course [2]. Folliculitis keloidalis nuchae (FKN) is a chronic, scarring folliculitis that leads to the development of keloid lesions that affect the occiput and the back of the neck of mostly black young males [3].

The objective of this study is to report a rare case of overlap of CVG, FD and FKN and discuss the possible common basis for these apparently coincidental associations.

Case Report

We report a 29-year-old black male with deep linear skin folds on the scalp, resembling the gyri of the brain, in an anteroposterior direction, on the temporoparietal regions. He had an area of scarring alopecia on the vertex with multiple papules, pustules and tufted hairs and keloidal scarring on the occiput. He had a history of use of dreadlocks for 5 years and noticed the scalp changes soon after removing them. His CT scan showed normal cranial contents and an extracranial mass caused by tumefaction of soft tissues in the temporoparietal regions. He was otherwise healthy. Culture of the pustules showed the presence of Staphylococcus aureus. Scalp biopsy was performed in three different areas: parietal area, vertex and occiput. The biopsies confirmed the diagnosis of CVG, FD and FKN, respetively. Clinical findings, dermoscopy and histopathology are summarized in figures 1, 2, 3, 4.

Fig. 1.

a Deep linear skin folds on the scalp, resembling the gyri of the brain, in an anteroposterior direction, on the temporoparietal regions. b Dermoscopy: no significant findings. c Histopathology: Sebaceous hyperplasia and dense hyalinized collagen.

Fig. 2.

a Scarring alopecia on the vertex with multiple papules, pustules and tufted hairs. b Dermoscopy: multiple upright hairs emerging from a single ostium and diffuse erythema. c Histopathology: intense mixed inflammatory infiltrate, with plasma cells, neutrophils and eosinophils, and atrophy of the follicular epithelium.

Fig. 3.

a Keloidal scarring on the occiput. b Dermoscopy: scarred hypopigmented areas with follicular paucity. c Histopathology: perifollicular fibrosis with mononuclear cell infiltration and loss of sebaceous glands.

Fig. 4.

a, b Cranial computed tomography: normal cranial contents, extracranial mass caused by tumefaction of soft tissues in the temporoparietal regions.

Discussion

Traction, chronical trauma and inflammation of soft tissues may be associated with cicatricial alopecias, such as FD and FKN, and with CVG [2,4]. According to Pujol's hypothesis, tufted hair folliculitis, observed in FD and FKN, is a secondary phenomenon that occurs following primary follicular damage and is caused by inflammatory cytokine secretion [5,6]. It has been hypothesized that cytotoxic proteins secreted by S. aureus, frequently detected in active lesions of FD and early stages of FKN, may act as superantigens and activate the secretion of inflammatory cytokines [7].

Chiarini et al. [8] studied if mechanisms known to be able to induce fibrosis were already present in the inflammatory phases of FD. The authors hypothesized that in genetically predisposed people, the infection of hair follicles by S. aureus induces an intense migration of neutrophils, recruited in the perifollicular/intrafollicular dermis by innate immunity mechanisms, such as IL-8. T lymphocytes, already present in the early stage, may be activated by microbial antigens (via processing by Langerhans cells) and superantigens (via the Vβ domain of the T-cell receptor) and release several proinflammatory (IFN-γ and TNF-α) and profibrotic mediators (TGF-β, b-FGF, IL-1β, and IL-4). Activated fibroblasts overproduce the extracellular matrix, the progressive accumulation of which leads to fibrosis [8]. The traction caused by the dreadlocks may facilitate scalp colonization by S. aureus. S. aureus superantigens may also be a trigger for the activation and secretion of inflammatory cytokines that may lead to collagen thickening.

The growth and development of hair follicles is influenced by a number of different growth factors and cytokines, particularly members of the fibroblast growth factor (FGF) family. Keratinocyte growth factor (also known as FGF-7) is a recently identified 28-kDa member of the FGF family that induces proliferation of a wide variety of epithelial cells, including keratinocytes within the epidermis and dermal adnexa. Yan et al. [9] showed that enhanced TGF-β1 expression and Smad3 phosphorylation were noted in keloid tissues, such as present in FKN.

Prolonged traction has been associated with the development of CVG in some cases, particularly in Sikh men [10,11,12]. In patients with acquired CVG, dermal collagen thickening is observed on pathologic review [13].

The pathogenesis of CVG is not yet known; nevertheless, some authors suggested an autosomal dominant condition caused by mutation in the FGFR2,3 because CVG is characterized by dermal hypertrophy [14]. FGFR2 gene encodes a transmembrane tyrosine kinase and can function as a mitogenic, angiogenic or inflammatory factor, depending on the cell type and/or the microenvironment [15,16].

Pachidermoperiostosis, a condition that is frequently associated with CVG, presents anomalies in fibroblast activity. According to Bianchi et al. [17], the epidermal growth factor system could therefore be involved in determining hypertrophy of the affected tissues.

The concomitant appearance of CVG, FD and FKN, 5 years after the use of dreadlocks, brings up the hypothesis that the dermal collagen thickening, a common feature observed in the three conditions, may be triggered by traction. Hyperproliferation of collagen, fibrosis and keloid lesions, common features observed in the three conditions, may be explained by the activation of the different FGF members. Table 1 resumes the main features of CVG, FD and FKN and the possible involved physiopathology associated with inflammatory changes and growth factor activation.

Table 1.

CVG, FKN and FD: clinical features, histopathology and physiopathology

	Clinical features	Histopathology	Physiopathology
CVG	Excessive growth of the scalp, with the formation of convoluted folds and furrows in a cerebriform pattern	Dermal collagen thickening; hypertrophy of the sebaceous structures; inflammation and edema	Mutation in the FGFR2 (not proved yet); epidermal growth factor system could be involved in determining hypertrophy of the affected tissues
FKN	Chronic scarring folliculitis that leads to keloid formation; fibrotic papules and nodules of the nape of the neck and the occiput; tufted hair	Chronic granulation tissue containing plasma cells, lymphoid cells and fibroblasts; late-stage: numerous thick bundles of sclerotic collagen	Enhanced TGF-β1 expression and Smad3 phosphorylation
FD	Papules and pustules that lead to scleroatrophic plaques; chronic, progressive course; tufted hair	Initial: acneiform infundibular dilatation and an intrafollicular/perifollicular infiltrate, composed mainly of neutrophil granulocytes; progression: the infiltrate becomes mixed, with neutrophils, lymphocytes, plasma cells, and foreign-body giant cells, and extends into the adventitial dermis; late-stage: follicular and adventitial dermal fibrosis	Cytotoxic proteins secreted by S. aureus, may activate the secretion of inflammatory cytokines; T lymphocytes, release several proinflammatory (IFN-γ and TNF-α) and profibrotic mediators (TGF- β, b-FGF, IL-1β, and IL-4); activated fibroblasts overproduce the extracellular matrix

Conclusion

The activation of FGF members in genetically predisposed patients, leading to collagen thickening and fibrosis, may be a common pathological basis for the development of CVG, FD and FKN in the same patient. We present a case of the coexistence of the three different scalp conditions. Although it seems to be a rare coincidence, the common background of traction as a possible main trigger of the three conditions points to the fact that in African-descent patients, trauma caused by the grooming habits and the use of hair extensions may be a frequent cause for the development of inflammation that can clinically be evident as one or more scalp abnormalities such as those presented in this work. The role of hair care practices in people of color and how it can influence the development of scalp diseases is particularly complex and may involve genetic predisposition [18,19].

The traction from the dreadlocks and the colonization of S. aureus may enhance inflammation contributing to the chronicity of the diseases. Further studies are needed to confirm the role of the growth factor family and of inflammation caused by traction and colonization with S. aureus in the genesis of hypertrophic and cicatricial scalp diseases.

Statement of Ethics

The patient provided informed consent for the publication of his case, and the study complies with human rights.

Disclosure Statement

The authors have no conflict of interest to declare.