Abstract
Introduction
In the field of hair transplantation (HT) surgery, transplantation techniques from autologous tissue dominate. The most widely used techniques are follicular unit transplantation, also known as strip technique, and follicular unit extraction.
Case Presentation
We report a case of an HT sequence of particular interest because of its unusual clinical presentation, chronic relapsing nature, and aggressiveness. The clinical presentation (fluctuating and communicating lesions in the occipital region), course, and symptomatology support the hypothesis of PCAS.
Discussion
PCAS or folliculitis dissecans or Hoffmann's disease is a rare disorder of unknown etiology. We speculate that the mechanical extraction of follicular units was the trigger. This case underlines the need for further studies as cases of PCAS may increase concomitantly with the increase in HT.
Keywords: Hair transplantation surgery, Perifolliculitis capitis abscedens et suffodiens, Follicular unit transplantation, Follicular unit extraction
Established Facts
In the field of hair transplantation (HT) surgery, transplantation techniques from autologous tissue dominate. The most widely used techniques are follicular unit transplantation and follicular unit extraction (FUE).
FUE is generally well tolerated. Postoperative pain and edema are minimal and easily managed. Folliculitis and pustules at the recipient site are common; involuntary subluxation of the FU grafts below the dermis may lead to cyst formation.
Among HT complications, there are no reported cases of perifolliculitis capitis abscedens et suffodiens (PCAS).
Novel Insights
We report a case of an HT sequence of particular interest because of its unusual clinical presentation, chronic relapsing nature, and aggressiveness.
Mechanical extraction of FUs was the trigger leading to immunological dysfunction and alteration of scalp microbiota in a context of a predisposition to the follicular occlusion tetrad.
This case underlines the need for further studies as cases of PCAS may increase concomitantly with the increase in HT.
Introduction/Literature Review
In the field of hair transplantation (HT) surgery, transplantation techniques using autologous tissue dominate [1]. The most widely used techniques are follicular unit transplantation (FUT), also known as strip technique, and follicular unit extraction (FUE) [1, 2].
While follicular unit transplantation involves excising strips of hair from the donor area and dissecting them into small follicular units (FUs), FUE involves harvesting individual follicular grafts with the help of a punch [1]. Neither technique is superior to the other [1, 2]. However one of the disadvantages of FUE lies in the blind harvesting of grafts, which is dependent on the instrumentation (cut and punch shape), operator strength, orientation, and size of the graft [2].
Nevertheless, FUE is generally well tolerated [2]. Postoperative pain and edema are minimal and easily managed [2]. Folliculitis and pustules at the recipient site are common in the first weeks and regress spontaneously without damaging the grafts [1, 2] Involuntary subluxation of the FU grafts below the dermis may lead to cyst formation [1, 2]. Another rare complication is necrosis resulting in scarring alopecia of the donor site [1, 2]. Among HT complications, there are no reported cases of perifolliculitis capitis abscedens et suffodiens (PCAS).
Case Presentation
We report a case of an HT sequence of particular interest because of its unusual clinical presentation, chronic relapsing nature, and aggressiveness. The patient came to our attention with a history of nodulocystic lesions for about 10 years, which arose 2 months after an FUE HT. The patient had no history of scalp folliculitis. The patient presented medical documentation of fluctuating cysts communicating with each other via fistulas draining pus and blood. Intense pain was associated. The lesions were repeatedly excised; however, new lesions were reforming in new areas of the occipital and vertex area. At clinical examination, the patient showed numerous cyst-induced scarring in the donor area (Fig. 1). Histological examination of the lesions reported an “Epithelial inclusion cyst surrounded by chronic granulomatous inflammation with multinucleated giant cells organized around hair shafts. The dermis is the site of diffuse fibrotic and periannexial changes. In the dermis, there is an abscessal collection with hair fragments escaping from a draining sinus.” Histology was compatible with an advanced stage of PCAS, although not pathognomonic and exclusive [3].
Fig. 1.
Patient's head: he presented to our attention with numerous scars.
The clinical presentation (fluctuating and communicating lesions in the occipital region), course, and symptomatology support the hypothesis of PCAS [4]. Indeed, our patient presented cysts in the donor area, whereas post-transplant cysts are typically in the recipient site, as the etiopathogenesis lies in the incorrect insertion of FUs with consequent formation of inclusion cysts and occurs in the weeks or months following surgery and does not persist for years [1, 2].
Discussion
PCAS or folliculitis et perifolliculitis abscedens et suffodiens or Hoffmann's disease is a rare disorder of unknown etiology; however, it is part of the so-called follicular occlusion tetrad along with hidradenitis suppurativa, acne conglobata, and pilonidal cyst [5]. Diagnosis is based on the presence of hard, fluctuating nodules and abscesses, especially at the occiput and vertex, often communicating with each other via fistulas draining pus and blood [4]. The course is chronic with multiple recurrences and marked scarring alopecia even after the resolution of the inflammatory process [4]. The pathophysiology is based on follicular occlusion with subsequent dilatation of the pilosebaceous canal [5]. Subsequently, the dissection of follicular acroinfundibulum causes inflammation and abscesses [6]. The primum movens toward follicular occlusion is still unknown; however, various mechanisms are hypothesized including mechanical destruction and/or immunological dysfunction of the affected hair follicle (either due to bacterial stimulation or activation of the innate immune response) [7]. We speculate that the mechanical extraction of FUs was the trigger leading to immunological dysfunction and alteration of scalp microbiota in a context of a predisposition to the follicular occlusion tetrad, though with unpredictable character [8]. This case underlines the need for further studies as cases of PCAS may increase concomitantly with the increase in HT.
Statement of Ethics
Ethical approval is not required for this study in accordance with local or national guidelines. Written informed consent was obtained from the patient for publication of the details of their medical case and any accompanying images.
Conflict of Interest Statement
None of the contributing authors have any conflict of interest, including specific financial interests or relationships and affiliation relevant to the subject matter or discussed materials in the manuscript.
Funding Sources
No funding was received.
Author Contributions
Lucia Genco and Mariateresa Cantelli: conceptualization, validation, visualization, writing − original draft preparation, and writing − review and editing. Mario Delfino and Matteo Noto: data curation, formal analysis, investigation, visualization, and writing − original draft preparation. Gabriella Fabbrocini and Marina Vastarella: conceptualization, validation, visualization, writing − review and editing, and supervision.
Data Availability Statement
All data generated or analyzed during this study are included in this article. Further inquiries can be directed to the corresponding author.
Funding Statement
No funding was received.
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Associated Data
This section collects any data citations, data availability statements, or supplementary materials included in this article.
Data Availability Statement
All data generated or analyzed during this study are included in this article. Further inquiries can be directed to the corresponding author.