Perifolliculitis capitis abscedens et suffodiens successfully controlled with topical isotretinoin

ARTICLE

Perifolliculitis capitis abscedens et suffodiens (otherwise known as dissecting folliculitis of the scalp or as dissecting cellulitis of the scalp or also as Hoffman's disease) is an uncommon, chronic, progressive, inflammatory, suppurative scalp disorder that almost exclusively affects Afro-Caribbean males. Spitzer in 1903 first described this entity (under the title Dermatitis Follicularis et Perifollicularis Conglobata) in association with severe acne conglobata. The second case report was presented by Nobl, before the Vienna Dermatological Society October 26, 1904, which fits into the clinical description of Hoffman's case. Hoffman presented the next case to the Berlin Dermatological Society on March 12, 1907 and named the disease perifolliculitis capitis abscedens et suffodiens [1]. It is a descriptive denomination (suffodiens: latin suffodio to dig under).

Interest in this clinical case presentation arises from the following data: (a) very few cases of dissecting folliculitis in white patients have been reported in the literature, (b) clinical features of this entity as well as the corresponding (to each case) histological picture may vary from case to case, (c) therapeutic means that have been applied and described in the literature are limited and frequently disappointing, (d) causes are unknown. Familial cases have been reported rarely [2, 3], (e) risk of malignant transformation of Hoffman's disease lesions has already been recognized [4, 5].

Case report

Our patient was a 20 year-old white male subject, presented at consultation because he sensed six months ago (and he has since observed) the existence (on the vertex region of the scalp) of two circumscribed, oval (of 3 cm in maximal diameter) alopecia plaques, in the central area of which a monofocal growth hair was left. Very small pustules were observed on the erythematous and inflammatory surface of these plaques. These alopecia areas are neighboring and a thin zone of normally growing hair was interposed between them (Fig. 1). We ascertained by palpation that very infiltrated and relatively yielding cystic formations, existed under the baldness plaques. A similar cystic formation, reminiscent of a sebaceous cyst, was palpated in the left-lateral area of the nape. The patient had mild microcystic and pustular acne on the face and the back. Before admission at our consultation, the patient had been managed by trials including systemic administration of antibiotics or corticosteroids as well as subcutaneous injection of corticosteroids. The above treatments had assured mild regression for 20 to 30 days and afterwards, a relapse (much more inflammatory compared to the preexisting clinical aspect) occurred.

Discussion

We presented above an 18 year-old male patient of phototype IV. Only 3 patients (among almost 50 cases analytically published in the literature) [1-23] concern subjects of the white race [10, 16, 21] (in 4 cases the race of the patients was not elucidated [9, 15, 16, 18] whereas all the other presentations included black patients). The majority of the patients are male (except 7 female subjects) [7, 11, 14, 15, 19]. First appearance of the disease age varies from 15 to 33 year-old, except one case concerning a lymphoma presenting as dissecting folliculitis [5]. It is worthwhile to report that the malignant transformation of the disease to squamous cell carcinoma, has been diagnosed at the age of 41 year-old (where dissecting folliculitis appeared 19 years previously) [4]. Clinically the disease begins as a folliculitis, most commonly involving the vertex and/or occipital scalp. This is rapidly followed by horizontal spreading, abscess, sinus formation and alopecia. Our patient presented at consultation in this second clinical evolutionary phase.

The whole process ends up with cicatrization and nodular formation. These nodules suppurate and spread to form intercommunicating sinuses. These sinuses, as long as 4 cm to 5 cm, can be easily traced by a probe. Seropurulent drainage may last indefinitely. The nodular form of five cases was associated with spontaneous severe pain [4, 10, 22, 23]. Sometimes a swelling of beard area may coexist.

The disease starts with a keratinized occluded follicular orifice. Stasis in the pilosebaceous apparatus promotes
fertile soil for bacterial multiplication. Folliculitis and perifoculitis results from follicular occlusion either from immune-immediate chronic inflammation resulting from hypersensitivity to Propionibacterium acnes or an immune complex disorder. Secondary infection probably ensues. Staph. aureus, staph. epidermidis, strept. beta-haemolytic, pseudomonas aeruginosa and diphtheroid bacilli may be isolated [4, 7, 8, 11, 12, 15]. However, bacteriologic cultures are usually negative [13, 18, 20-23].

The younger lesions showed a benign hyperkeratosis around the follicle orifice, atrophy of the orifice, massive keratotic plugging and an infiltrate (in the upper dermis) consisting primarily of neutrophils. Lesions of longer duration had an infiltrate of plasma cells, histiocytes, lymphocytes in the upper and middle corium. Older lesions showed predominantly plasma cells [11, 12, 15, 23]. A foreign body giant cell reaction and granulomatous tissue formation are observed in the abscess-alopecia second stage of the disease (as seen in our case) as well as in the nodular-cicatrizant phase. The giant cells frequently had material in them, which appeared to be keratin. There were changes of liquefaction degeneration of the collagen and elastic tissue around the areas of inflammation [4, 6, 7, 9, 14, 15, 21]. Combination with acne vulgaris is common (in 30 % of the cases). The disease is considered to be part of the follicular occlusion tetrad, together with acne conglobata, chronic hidradenitis suppurativa and pilonidal cysts [8, 24]. The central pathogenic event in all four entities is a tendency toward follicular hyperkeratosis leading to retention of follicular products with secondary bacterial infection. The common anatomoclinical features of the follicular occlusion are the following: formation of multiple comedones, abscesses with communicating channels, discharging sinuses.

Squamous cell carcinomas may arise within acne conglobata and hidradenitis suppurativa. One case in which a metastasizing squamous cell carcinoma developed in a long-standing lesion, has already been reported [4]. An aggressive large cell variant of folliculotropic mycosis fongoïdes should be included in the differential diagnosis of dissecting cellulitis of the scalp, emphasizing the importance of a biopsy in any nonhealing lesion, to rule out lymphoma [5]. Cutaneous signs of follicular occlusion may coexist with asymmetric peripheral and axial arthritis [7, 21, 22].

Treatment is varied and generally unsuccessful. Oral antibiotics and oral zinc have occasionally produced good results [16]. Satisfactory results using x-ray epilation have been reported but radiation therapy for benign conditions is avoided because of potential long-term adverse effects [14]. Systemic or intralesional steroids provide only partial relief, moreover might favor eventual malignant transformation [15, 20]. Oral isotretinoin is effective, although it needs to be continued for 4 months after clinical control is achieved, to prevent relapse [2, 16, 17, 22, 23]. Oral isotretinoin has been proven sufficiently efficacious as monotherapy [23], in the abscess-alopecia second stage whereas oral antibiotics and/or systemic (or intralesional) steroids have been required (as combination therapy with POs retinoids) in the nodular-scarring third stage of the disease [22]. A surgical approach may be employed in resistant nodular cases. The lesions are incised and drained. It is possible to pass a probe through burrows of interconnecting pustular nodules. Intercommunicating sinuses should be marsupialized and the surface is cauterized to destroy the epithelium lining the sinuses [18]. Removal of the entire scalp with subsequent grafting has been published [12].

CONCLUSION

In our case the topical isotretinoin and clindamycin blocked the disease in its second phase and thus inhibited the evolution to scarring and nodular stage. Clindamycin has antibacterial and antiinflammatory action [25]. Topical isotretinoin (as well as the topical retinoic acid or the adapalene) is characterized by antiinflammatory activity as well as desquamative and keratolytic action at the follicle infundibulum. Hair regrowth was eventually favored by the inhibitory retinoid action concerning the enzymatic 5a-reductase activity [26, 27]. Therefore as already supposed in hidradenitis suppurativa [28, 29], hormonal etiopathogenetic mechanisms are suspected in dissecting folliculitis and have to be looked for in the future. Moreover continuous isotretinoin topical application might protect from eventual malignant transformation [30].

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