A Very Rare Case of Dissecting Cellulitis of the Scalp in an

Indonesian Man

Rizky Lendl Prayogo

, Lusiana

, Sri Linuwih Menaldi

, Sondang P. Sirait

, Eliza Miranda

Department of Dermatology and Venereology Faculty of Medicine Universitas Indonesia / Dr. Cipto Mangunkusumo

National General Hospital, Jakarta, Indonesia

Keywords: dissecting cellulitis of the scalp, dissecting folliculitis, follicular occlusion tetrad, diagnosis, isotretinoin

Abstract: Dissecting cellulitis of the scalp (DCS), also known as dissecting folliculitis, perifolliculitis capitis abscedens

et suffodiens (PCAS), or Hoffman’s disease, is a primary neutrophilic cicatricial alopecia without clear

etiology. Along with hidradenitis suppurativa, acne conglobata, and pilonidal cyst, they were recognized as

‘follicular occlusion tetrad’. A 43-year-old Indonesian man presented to our department with four years

history of persistent, slightly painful subcutaneous nodules, abscesses, and sinuses that discharged purulent

exudate on vertex and occipital scalp. There was also associated patchy alopecia. He had severe acne during

his adolescence to early adulthood. Trichoscopic evaluation showed yellowish and whitish area lacking of

follicular openings. Histopathological examination showed follicular occlusion, dilatation, and rupture with

mixed inflammatory infiltrates, mainly neutrophils. The diagnosis of DCS was confirmed by clinical,

trichoscopic, and histopathological examinations. Isotretinoin 20 mg daily was given to normalize the

follicular keratinization. Considering its very rare occurrence in an Indonesia man, this case was reported to

emphasize the diagnosis of DCS.

1 INTRODUCTION

DCS, also known as dissecting folliculitis, PCAS,

or Hoffmann’s disease, is a very rare primary

neutrophilic citatricial alopecia without clear etiology

(Badaoui et al., 2016). It was first described by

Spitzer in 1903 who termed the disease “dermatitis

follicularis capitis et perifollicullaris

conglobata”(Spitzer, 1903). Hoffman then termed it

as PCAS in 1908 (Hoffman, 1908). Since the first

description to 2014, the details of only 72 patients

have been published.1 DCS has been considered to be

a part of ‘follicular occlusion triad’, along with

hidradenitis suppurativa and acne conglobata (Otberg

& Shapiro, 2012). Other literature included pilonidal

cyst and altogether they were recognized as ‘follicular

occlusion tetrad’ (Badaoui et al., 2016). Its chronic

relapsing courses resulted in cicatricial alopecia with

hypertrophic or keloidal scars formation (Otberg &

Shapiro, 2012) . Various treatments, such as systemic

antibiotics, intralesional corticosteroid, oral

prednisolone, and isotretinoin showed clinical

improvement (Otberg & Shapiro, 2012; Scheinfeld,

2014). In refractory and more advanced cases, anti

tumor necrosis factor alpha (anti-TNF α) and surgery

should be considered (Otberg & Shapiro, 2012;

Scheinfeld, 2014). Considering its low prevalence in

Indonesia, we are intrigued to report a case

emphasizing the diagnosis of DCS.

2 CASE

A 43-year-old Indonesian man presented to our

department with four years history of persistent,

slightly painful lumps that discharged purulent

material during compression on his vertex and

occipital scalp. There was also associated patchy

alopecia. Those lumps firstly appeared as small red

bumps resembling folliculitis, which then enlarged.

He experienced severe facial acne during his

adolescence to early adulthood. He regularly washed

his hair daily and got his hair cut with scissors

monthly. He often wears a hat, which was washed

once every one or a couple of weeks. There was no

familial history with the same complaints and

previous mechanical trauma. On physical

examination, there were multiple flesh-colored

subcutaneous nodules that fluctuated, sinuses that

discharged purulent exudate, and patchy alopecia.

398

Prayogo, R., Lusiana, ., Menaldi, S., Sirait, S. and Miranda, E.

A Very Rare Case of Dissecting Cellulitis of the Scalp in an Indonesian Man.

DOI: 10.5220/0008158303980401

In Proceedings of the 23rd Regional Conference of Dermatology (RCD 2018), pages 398-401

ISBN: 978-989-758-494-7

These lesions were slightly painful (VAS 2-3). The

surrounding tissues were neither erythematous nor

edematous. We also did not observe any tufted hairs.

Trichoscopic evaluation showed yellowish and

whitish area lacking of follicular openings.

Microscopic examination with KOH 20% from the

alopecia patch revealed no fungal elements. Bacterial

culture from the discharge and skin tissues were done

and showed growth of Staphylococcus epidermidis,

which was still sensitive to various antibiotics.

Histopathological examination showed follicular

occlusion, dilatation, and rupture with mixed

inflammatory infiltrates, mainly neutrophils. Based

on the clinical, trichoscopic, and histopathological

examinations, the diagnosis of DCS was confirmed.

Isotretinoin 20 mg daily was then initiated.

Figure 1. Clinical lesions: A: Fluctuating flesh-colored

nodules in vertes-occipital scalp area; B: draining sinuses

with purulent discharge.

Figure 2. Trichoscopic and histological examination. A:

Yellowish and whitish are lacking of follicular openings; B:

Follicular occlusion and dilatation (H&E40x).

3 DISCUSSION

Dissecting cellulitis of the scalp is a very rare disease

characterized by inflammative nodules, abscesses,

and sinuses, which may progress into scarring

alopecia (Segurado-Miravalles et al., 2017). Along

with hidradenitis suppurativa, acne conglobata, and

pilonidal cyst, DCS forms the follicular occlusion

tetrad (Badaoui et al., 2016;Segurado-Miravalles et

al., 2017). These diseases shared similar

etiopathogenesis involving hyperkeratosis, follicular

occlusion, and subsequent inflammation (Segurado-

Miravalles et al., 2017). Not all patients presented all

these four diseases (Badaoui et al., 2016). A

retrospective study of 51 patients showed that 12%

patients were also presented with hidradenitis

suppurativa, 16% with acne conglobata, and 4% with

coexisting hidradenitis suppurativa and acne

conglobata (Badaoui et al., 2016). No patients were

identified as having pilonidal cyst (Badaoui et al.,

2016). Mechanical trauma as a predisposing factor

was only found in five patients (Badaoui et al., 2016).

Subjective complains are pain or itch (Badaoui et al.,

2016). Eighty six percent patients progressed into

chronic disease (Badaoui et al., 2016). The observed

abscesses were usually sterile (Gaopande et al.,

2015). Bacterial infection can occur secondarily

during the course (Gaopande et al., 2015).

Microorganisms cultured in reported cases include

Pseudomonas species, Staphylococcus epidermidis,

Prevotella intermedia, Peptostreptococcus

asaccharolyticus, and Proprionibacterium acnes

(Gaopande et al., 2015). DCS is predominantly found

in African American men between 20 and 40 years

(Gaopande et al., 2015). Although it rarely affected

Asian, it has been reported in other Asian ethnicities

(Chinese and Indian) (Gaopande et al., 2015; Qi et al.,

2014). A retrospective study by Badaoui et al Badaoui

et al., 2016). showed that the mean age was 26,6

years, with wider range of age between 15-62.

Gaopande et al (2016) even reported the occurrence

of this disease in a 7-year-old boy. A multicenter

study which was conducted in four hospitals in Spain

also reported the occurrence in a 23-year-old Asian

woman (Segurado-Miravalles et al., 2017). The

pathophysiology of DCS remains unclear (Badaoui et

al., 2016). The young age of onset, the occurrence in

patients with dark phototype, and the cases of familial

DCS suggest a genetic predisposition (Badaoui et al.,

2016). The predominance of patients with dark

phototype raises the question of the role of hair type,

which is often coarse and frizzy, as well as traumatic

factor, such as hair shaving (Badaoui et al., 2016).

The male dominance and vertex as the predilection

A Very Rare Case of Dissecting Cellulitis of the Scalp in an Indonesian Man

399

area could also suggest a hormonal risk factor

(Badaoui et al., 2016). Finally, commensal bacteria

may play an essential role as alloantigens in the

pathophysiology of DCS (Badaoui et al., 2016). The

loss of immune tolerance to these alloantigens may

lead to an inflammatory reaction (Badaoui et al.,

2016; Scheinfeld, 2014). To the best of authors’

knowledge, this is the third case of DCS in an

Indonesian man. Although unpublished, Sirait (2015)

reported the occurrence of DCS associated with

hidradenitis suppurativa in 2015. Rahman et al.

(2017) then reported a case of follicular occlusion

tetrad in 2017. This patient is a 43-year-old

Indonesian man with straight hairs and no associated

mechanical trauma. There was also no familial

history with the same complaints. The signs and

symptoms of patient reported in this report are well

suited with DCS despite the lack of associated

predisposing factors except male gender. Other

explanations supporting the diagnosis of DCS are

listed below:

1. Anamnesis: lumps that discharged purulent

material on the predilection areas. History of

severe acne during his adolescence to early

adulthood.

2. Physical examination: observed subcutaneous

nodules, abscesses, and sinuses that

discharged purulent exudate.

3. Trichoscopic examination: yellowish and

whitish area lacking of follicular openings.

These were consistent with trichoscopic

findings explained by Laccarubba et al (2017).

4. Histopathological examination: follicular

occlusion, dilatation, and rupture with mixed

inflammatory infiltrates, mainly neutrophils.

Multiple hair shaft fragments are evident in

pilonidal cyst (Calonje et al., 2012). This

feature is not observed, so that the

histopathology is more suited with DCS than

pilonidal cyst.

DCS must be distinguished with other diseases

involving scalp (Vasant et al., 2014). The tendency of

DCS to cause fluctuating nodules and sinus tracts

helps to distinguish it from acne keloidalis nuchae

(AKN) (Vasant et al., 2014). Folliculitis decalvans,

another differential diagnosis, is characterized by

tufted folliculitis, in which multiple hair tufts emerge

from dilated follicular orifices (Vasant et al., 2014).

Various therapeutic strategies were reported

successful in treating DCS, such as systemic

antibiotics (minocycline, tetracycline, cloxacillin,

erythromycin, cephalosporin, or clindamycin),

intralesional corticosteroid, and oral prednisolone

(Otberg & Shapiro, 2012). The benefits of systemic

antibiotics were considered to be their anti-

inflammatory effects rather than antibacterial (Otberg

& Shapiro, 2012). Isotretinoin 0,5-1 mg/kg/day has

shown prolonged remission (Otberg & Shapiro,

2012). The mechanisms of action of isotretinoin are

normalizing follicular keratinization and reducing the

aberrant immune responses (Scheinfeld, 2014). Anti-

TNF α may be used when isotretinoin fails

(Scheinfeld, 2014). It can also defer the need of a

surgical treatment (Scheinfeld, 2014). Incision and

drainage may be done to painful and resistant nodules

(Scheinfeld, 2014). Marsupialization with curettage

of the cyst wall and total scalp excision followed by

split-thickness skin grafting have been reported, but

these surgical procedures should only be done for

extreme and refractory cases (Otberg & Shapiro,

2012). Badaoui et al. (2016) reported that 78%

patients receiving systemic antibiotics (doxycycline,

pristamycin, rifampicin, or a combination of several

antibiotics) showed moderate improvement.

However, the disease was relapsed in all of those

patients after antibiotic cessation (Badaoui et al.,

2016). Seventy one percent patients had received

systemic retinoid and almost all (92%) showed

complete remission after 3 months (Badaoui et al.,

2016). Isotretinoin is considered as the first line

therapeutic option for DCS (Badaoui et al., 2016;

Fransisco et al., 2017). The efficacy of isotretinoin in

treating DCS has been published (Marquis et al.,

2017). However, the optimal dose, duration of

therapy, and combination with other agents have not

been fully elucidated (Marquis et al., 2017). Marquis

et al.(2017) reported an excellence therapeutic

response after 4 months course of isotretinoin at 0,27

mg/kg/day. Considering the possibility of developing

side effects given at higher dose, this patient received

isotretinoin 20 mg daily (equal to 0,25 mg/kg/day).

He has been receiving isotretinoin for the past two

months and showed improvement.

4 CONCLUSIONS

DCS is a very rare case in Indonesia and should be

recognized as one of differential diagnosis when

seeing nodules, abscesses, and sinuses on the scalp.

The diagnosis can be confirmed by clinical,

trichoscopic, and histopathological examinations.

The therapy can be started immediately after the

diagnosis has been confirmed.

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400

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