Are You Confident of the Diagnosis?
Perifolliculitis abscedens et suffodiens was first described by Spitzer in 1903 and then aptly so named by Hoffmann in 1908. The first American reports were by Wise and Parkhurst in 1921. Though Gray noted the complementary appearance of acne conglobata and perifolliculitis in 1931, it was not until Kierland in 1951 and Pillsbury in 1956 when the pathogenic grouping of this still poorly understood rare disease was considered a member of the “follicular occlusion triad” of acne conglobata, hidradenitis suppurativa and perifolliculitis. Some authors have added pilonidal sinus, which is really structural as a tetradic member.
Less than 100 cases have been reported in the literature of those, the largest number are male (80%) and most patients are of Black African ancestry.
Characteristic findings on physical examination
The disease is clinically characterized by painful, rarely itchy, recurrent firm to fluctuant spongy nodules, which may be from 1 to 4cm, often coalescing into sausage forms (Figure 1). The nodules may spontaneously break with purulent, often foul-smelling drainage. Pressure on one pole will often express pus at a distal pole (revealing extensive undermining or even sinus tract formation) or even from follicular orifices. The surface of a nodule is red and shiny and loses its hair. Scaling is not characteristic nor is cervical lymphadenopathy, although the latter may occur. Follicular plugging may occur in areas of alopecia.
Some authors have described discrete, even numerous pustules; even confluence into lakes of pus may be observed. These features may reflect an overlap or confusion with folliculitis decalvans. The area affected is the vertex and occiput but the entire scalp may be involved. Nodules of acne conglobata may occur on the adjacent face and neck. The nodules may heal with atrophic dyschromic scarring and patchy alopecia. Islands of tufted hair, as seen in folliculitis decalvans, may be observed.
If therapy is successful there may be complete regrowth on areas devoid of hair. Though part of a triad, there may be no co-existent acne or hidradenitis. There is no true hot confluent edematous cellulitis, so the name “dissecting cellulitis” is misleading. Bacteriologic studies of drainage swabs or even biopsies usually reveal no aerobic bacterial pathogens, no dermatophytes or deep fungi or mycobacteria.
Probable secondary bacterial infection with coagulase negative or coagulase positive Staphylococcus aureus, Pripriionbacterium acnes, even Pseudomonas has been reported. Anaerobic cultures are not usually performed but when done have revealed Prevotella intermedia and Peptostreptococcus asaccharolyticus, both sensitive to clindamycin.
Expected results of diagnostic studies
Cultures of the drainage, but also of the patient’s hairbrush or a vigorous toothbrushing of the surface, must always be done to rule out Trichophytic infection (esp T tonsurans). If tinea capitis is considered as a possible alternate diagnosis, underdosing with griseofulvin in children may mask the diagnosis and response—a proper dose of 20 to 25mg/kg for at least 6 weeks should have been given before calling the nodules perifolliculitis in face of negative cultures.
Full blood counts, SMA-6 and SMA-12, serum immunoglobulins, complement levels, neutrophil function tests, peripheral lymphocyte immunophenotyping, serum zinc levels are usually entirely normal. The erythrocyte sedimentation rate (ESR) may be elevated. If there is concurrent arthritis, rheumatoid factor (RF) and antinuclear antibody (ANA) should be performed. HLAB27 is usually negative even in this instance since this genetic marker is rare in African-Americans.
Histologic examination depends on the age of the lesion selected. Early small lesions may show acneiform distention (keratotic plugging) of the follicular infundibula with intrafollicular and perifollicular neutrophilic infiltrates. There may be some follicular hyperkeratosis around the orifice. Special stains for fungus, bacteria and mycobacteria are negative. Later, deep-seated abscesses develop in the dermis and subcutis; response to keratinous debris incites a granulomatous response with foreign body giant cells and a more mixed chronic infiltrate of plasma cells, histiocytes and lymphocytes even eosinophils as well as neutrophils.
Late-stage lesions will show scarring, sometimes massive in those with a clinical picture of keloidal-type scar and sinus tracts, partly lined with squamous epithelium. Such exuberant scar and tract formation or pseudocyst entrapment is not unique and can be seen in folliculitis papillaris as well.
Alopecic and aseptic nodules of the scalp is a recently described entity appearing as 1 to 6 firm rarely fluctuant nodules mostly on the occiput, but also possibly involving the parietal scalp, and even the vertex and beard in young, predominantly non-Black males (Caucasians and Asians are described). Cultures are negative but the nodules respond with clearing and regrowth of hair without scarring to doxycycline 100mg daily within 3 months, whereas patients with perifolliculitis may improve but do not clear in 3 months. Histologically the dermal infiltrate was diffuse with a more destructive process than perifolliculitis and involving the whole dermis.
Firm nodules, representing the very rare development of aggressive metastasizing squamous cell carcinoma in perifolliculitis, should be looked out for. Similarly in patients with the very rare KIT syndrome, aggressive malignant proliferative cysts should be searched for repeatedly.
Follicular mycosis fungoides can also present with chronic nonhealing draining nodules of the scalp mimicking perifolliculitis. Skin biopsy should always be performed in perifolliculitis to help rule out this rare condition as well.
Who is at Risk for Developing this Disease?
This disease is so rare that it is impossible to determine who is at risk except for those already afflicted by portions of the occlusion triad, particularly if they are black males. Even so, since all elements of the triad are not evident in all patients, having one element should not be a concern of invariable harbinger of perifolliculitis.
What is the Cause of the Disease?
The cause is unknown but thought analogous to acne vulgaris by virtue of abnormal keratinization of the follicular orifice and infundibulum, which induces follicular occlusion and a cascade of inflammatory elements from follicular breakdown products and P acnes.
Systemic Implications and Complications
HLA B27-negative erosive and proliferative peripheral as well as axial arthropathy can be seen on radiologic examination of symptomatic patients. polyarthralgia may precede or follow the scalp disease. The most feared complications of perifolliculitis are calvarial osteomyelitis (which should be ruled out by computed tomography (CT) exam in severely symptomatic patients, particularly if a surgical scalping or marsupialization is entertained) and malignant degeneration to squamous cell carcinoma.
Marjolin’s ulcers at the site of chronic inflammation (ie, invasive squamous cell carcinoma), which have a very aggressive potential and may metastasize occultly and early, have been reported to arise in acne conglobata and hidradenitis suppurativa. Only one case so far has been reported to occur in perifolliculitis. Two similar cases of malignant proliferating cysts of the scalp with perifolliculitis in KID syndrome (sensorineural hearing loss, photophobia, palmoplantar keratoderma, and recurrent infection).
Sudden appearance of firm, growing, irregular nodules in the sea of inflammatory nodules warrants immediate or repeated biopsies.
Other oral agents
–ILCS: triamcinolone acetonide 4 to 40mg/cc
–Incision and drainage
–Excision of nodules
–Marsupialization of sinus tracts
–Carbon dioxide laser marsupialization
–X-ray epilation: Kienbock-Adamson technique
–Electron beam epilation
–Nd:YAG laser long pulse
Optimal Therapeutic Approach for this Disease
There is no one optimal approach for all patients. Simple disease may require simple antibiotics and drainage as in acne. The disease, however, can be rapidly progressive and disabling, forcing one’s hand into using isotretinoin, oral prednisone or even biologics right away to control the disease. Longstanding disease with “architectural deformity” (ie, sinus tract formation) requires eventual aggressive surgery as no anti-inflammatory program will remove this nidus for further flares.
For mild simple disease then use of incision and drainage, Intralesional triamcinolone of usually 3 to 5mg/cc dosing and anti-inflammatory antibiotics such as minocycline or doxycycline 100mg orally twice a day or cipro 500mg twice a day may be adequate. In view of published data, innocuous use of short-term zinc sulfate 400mg orally 3 times a day, even in the face of normal serum zinc, may help resolution as early as 3 weeks.
Combination therapies of oral agents such as antibiotics plus rifampin and/ or zinc sulfate are not unreasonable, as long as they do not interfere with proper absorption. They would seem more amenable to treating secondary infection rather than the primary inflammation alone. For rapid control of inflammation, use of oral prednisone should be considered as well as intralesional injections, which are more limited by their dispersion. Combinations of doxycycline plus prednisone have been reported at least temporarily effective.
Isotretinoin partial remission may be seen as early as 1 month at 0.85mg/kg but even as low a dose as 10mg orally daily has been touted as effective. In an isotretinoin course it may take many months for all the nodules to shrink, and therapy may last a full year, unlike the usual 20-week course for severe acne; however, relapse may occur after further months to years as the architecture is unchanged by the medication. Some patients require multiple repeat courses of isotretinoin, but surgery should be performed after the first course to prevent this recurrence. Combination therapy of isotretinoin 80mg orally daily with dapsone 100mg daily has been claimed successful.
Biologics are said to be very effective and quickly effective in producing a remission—etanercept, infliximab and more recently adalimumab have been successfully employed in the triad. The problem is not rapidity but duration and very high cost of continued therapy. For instance, adalimumab, administered as 80mg subcutaneously, then 40mg every 2 weeks, yields a favorable response within 8 weeks but a relapse occurs if it is discontinued for 4 weeks; histologically there is much less post-treatment inflammation but no removal of prior sinus tracts. With maintenance every 2 weeks, hair begins to regrow by 5 months.
For infliximab at 5mg/kg every 8 weeks for 1 year—hair regrowth began as soon as 1 month with at least a 1-year remission.
Experience with remissions from X-radiation therapy in epilating doses made “removal of hair” a viable concept for laser surgeons, even though the presence of hair has never been considered a triggering factor here, unlike central centrifugal cicatricial alopecia (CCCA) or acne keloid. Perhaps follicular damage from laser-induced heating interferes with the abnormal keratinization or cytokine release, theoretically explaining its beneficial effect. Hair has no presence, most often, nor role in acne conglobata nor hidradenitis, the other triad partners.
Diode laser at 810nm, 30millisec dose at 29 J/cm2 over four sessions succeeded in a black male, though he clinically more resembled folliculitis decalvans with a mostly pustular appearance. Long-pulse 694nm ruby laser at 10 to 23J/cm2 with 10mm spot size over four courses was reported as therapeutic. Long-pulse 1064 nm Nd:YAG laser after topical occluded anesthesia or ring block local anesthesia was used without cooling at 20 to 50J/cms and pulse widths from 40 to 70millisec through Saran wrap, with a spot size of 10mm, and repeated over 1-year duration in 3 to 7 monthly sessions, with good control. These settings did not result in total depilation. A patchy but significant hair presence required a shaved head for cosmesis.
Radiation therapy is still a double-edged sword, since most patients are in their 20s or 30s; there is reluctance to use radiation with a long lag time, because of potential development of squamous cell carcinoma, or even basal cell carcinoma, decades later. Kienbock-Adamson (a-five point radiation technique covering the scalp) has been rarely reported as carcinogenic years after epilating treatment for tinea capitis. The success rate is admirable in perifolliculitis with pain and discharge improving literally within days to weeks. Epilating doses means that the normal hairs in unaffected areas will regrow and regrowth will appear on the previously bald remitted nodule sites. Mild relapse can occur months later, especially if parameters did not produce a totally epilating dose.
Patient management is a stepwise approach understanding that a rapidly explosive condition may ensue so close bimonthly to monthly followup is required for initially milder cases. Treating as one would treat moderate to severe acne is initially appropriate, but a rapid clinical deterioration should encourage a much more aggressive, early therapy with oral prednisone, isotretinoin, or biologics over a period of weeks to months. Radiation therapy must be discussed with a knowledgable radiotherapist. Once nodules have flattened, any obvious areas of sinus formation must be excised, or preferably marsupialized after adequate probe exploration for tracts intraoperatively, and then left to granulate and heal. Split-thickness grafting should be saved for those who may require total scalp removal (which may be necessary); here the disease is eliminated but the repair remains very deforming and will require a hairpiece.
Unusual Clinical Scenarios to Consider in Patient Management
Biopsies and cultures should be considered in any cases that are in any way atypical. For example, follicultropic cutaneous T cell lymphoma has presented as dissecting cellulitis as have tinea infections.
Vigilance must be maintained for the possiblity of secondary non-melanoma skin cancers within long-standing lesions.
What is the Evidence?
Berne, B, Venge, P, Olhman, S. . “Perifolliculitis capitis abscedens et suffodiens (Hoffman) complete healing associated with oral zinc therapy )”. Arch Dermatol. vol. 121. 1985. pp. 1028-30. (Unique anecdotal but well-studied dramatic rapid clearing of inflammatory and architecturally distorted disease with at least 5-year remission.)
Chinnaiyan, P, Tena, L, Brenner, M, Welsh, J. “Modern external beam radiation therapy for refractory dissecting cellulitis of the scalp”. Br J Dermatol. vol. 152. 2005. pp. 777-9. (Detailed treatment parameters on four patients with refractory perifolliculitis treated with electron beam or electrons and photons up to 35Gy with 4- to 13-year remissions of disease but eventual partial recovery of hair.
Curry, S, Gaither, D, King, L. “Squamous cell carcinoma arising in dissecting perifolliculitis of the scalp. A case report and review of secondary squamous cell carcinomas”. J Am Acad Dermatol. vol. 4. 1981. pp. 673-8. (Rapidly fatal metastatic squamous cell carcinoma of scalp in a black male with many years' history of perifolliculitis but also other elements of occlusion triad.)
Khaled, A. “Dissecting cellulitis of the scalp: response to isotretinoin”. JEADV. vol. 21. 2007. pp. 1430-1. (Patient required 1 year of therapy at 0.8mg/kg or–total dose 170mg/kg)
Klein, SL, Miller, MH, Kumar, P, Henschke, U, Kovi, J. “Perifolliculitis capitis abscedens et suffodiens”. Nat Med Assoc. vol. 68. 1976. pp. 9-13. (Intriguing discussion of various techniques of epilation by radiation devices with remission only after total temporary epilation.)
Moschella, SL, Klein, MH, Miller, RJ. “Perifolliculitis capitis abscedens et suffodiens. Report of a successful therapeutic scalping”. Arch Dermatol. vol. 96. 1967. pp. 195-7. (The classic ”excision of bad architecture” article.)
Moyer, D, Williams, R. “Perifolliculitis capitis abscedens et suffodiens”. Arch Dermatol. vol. 85. 1962. pp. 118-124. (Midcentury review of six cases showing technique and benefit of Kienbock-Adamson type of x-radiation combined with tract excision. Sterile cultures overwhelmingly predominant.)
Navarini, A, Trueb, R. “3 cases of dissecting cellulitis of the scalp”. Arch Dermatol. vol. 146. 2010. pp. 517-20. (Three cases of recalcitrant disease with successful resolution within 8 weeks but relapse within 1 month of discontinuation. Surgical excision of distorted architecture may be a necessary adjunct.)
Ramos e Silva, J. “Peri-foliculite abscedante de Hoffmann – sua possível etiologia micótica”. Hospital, Rio Janeiro. vol. 50. 1956. pp. 767-80. (Tricophyton species)
Wise, F, Parkhurst, H. “A rare form of suppurative and cicatrizing disease of the scalp (perifolliculitis capitis abscedens et suffodiens)”. Arch Dermatol. Syph. vol. 4. 1921. pp. 751(First American description)
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