Dissecting cellulitis of the scalp
Published Web Locationhttps://doi.org/10.5070/D35v6848mq
Dissecting cellulitis of the scalpThe Ronald O. Perelman Department of Dermatology, New York University School of Medicine, New York, New York
Jyoti P Mundi MD, Shoshana Marmon MD PhD, Max Fischer MD, Hideko Kamino MD, Rishi Patel MD, Jerry Shapiro MD
Dermatology Online Journal 18 (12): 8
Dissecting cellulitis of the scalp is a chronic, relapsing, inflammatory disease of the scalp that results in scarring alopecia. We present a case of a 32-year-old man with recalcitrant disease who is now responding to treatment with isotretinoin. The pathogenesis, clinical presentation, disease associations, and histopathological findings are reviewed. Treatment can be challenging. The literature on medical and surgical therapeutic options is reviewed.
The patient is a 32-year-old man with patches of hair loss and open draining sores on the scalp. He has been treated with topical and intralesional glucocorticoids for five years and oral antibiotics in the past without notable improvement.
Our patient received a one-week course of cephalexin to treat MSSA that grew out of wound cultures. Isotretinoin 1 mg/kg/day was prescribed and after approximately one month of treatment, the patient notes improvement in the level of drainage.
Boggy, fluctuant, draining tracts, multiple erosions, and patches of alopecia were present on the scalp, most prominently in the occipital area.
Fungal culture showed no growth, Wound Gram stain showed rare neutrophils, few Gram positive cocci in pairs, and a few yeasts. Wound culture showed numerous methicillin sensitive Staphylococcus aureus.
There is a superficial and deep perifollicular suppurative and granulomatous infiltrate associated with lymphocytes and plasma cells around a cystically dilated, partially disrupted hair follicle. Gram-positive cocci are present within the hair follicle. A periodic acid-Schiff with diastase stain fails to reveal fungi.
Dissecting cellulitis of the scalp, which is known as perifolliculitis capitis abscedens et suffodiens or Hoffman disease, is a chronic, relapsing disease of the scalp that, if left untreated, eventuates into scarring alopecia. The disease begins as a follicular pustule on the occipital or vertex scalp that transforms into a painful, fluctuant nodule. Multifocal disease is common. Burrowing interconnecting abscesses may impart a cerebriform, boggy appearance to the scalp. Although the exact pathogenesis is unknown, the disease is associated with acne conglobata and hidradenitis suppurativa, which are collectively referred to as the follicular occlusion triad. This association suggests a common pathogenic mechanism of abnormal follicular keratinization and occlusion, secondary bacterial infection, and follicular destruction, with a subsequent neutrophilic and granulomatous inflammatory response .
Although the disease most commonly affects black men in their second to fourth decades, 10 percent of cases involve white men and it has been rarely reported to occur in women and children. Coexisting acne conglobata or hidradenitis suppurativa is present in approximately one-third of patients and is a risk factor for the development of human leukocyte antigen-B27-negative spondyloarthropathy, especially in black men. This arthropathy is characterized by an asymmetric peripheral and axial joint arthritis. Polyarticular arthritis with sternoclavicular hyperostosis, SAPHO syndrome (synovitis, acne, palmoplantar pustulosis, hyperostosis, osteitis), marginal keratitis, pyoderma vegetans, and pityriasis rubra pilaris  have been reported to occur in association with dissecting cellulitis. Additionally, osteomyelitis and metastatic squamous-cell carcinoma have been reported as sequelae .
The histopathologic features vary according to the stage of disease: early lesions are characterized by acneiform distention of the follicular infundibula with a perifollicular, mixed, neutrophilic, and lymphoplasmacytic inflammation. The lower portion of terminal follicles is most affected. Some follicles enter catagen/telogen phases of the hair cycle, which results in shedding of hair shafts without follicular destruction. At this stage, treatment may result in hair re-growth in affected areas. As inflammatory cells invade the follicular epithelium, follicular destruction ensues and deep-seated abscesses form in the adventitial dermis and subcutis. Sinus tracts that are partly lined with squamous epithelium and surrounded by dense fibrosis characterize late-stage lesions [3, 4].
Both medical and surgical therapeutic alternatives have been applied although data are limited to case reports and small series. Isotretinoin is advocated as first-line therapy at an initial dose of 1 mg/kg/day, which is followed by a maintenance dose of 0.75 mg/kg/day once clinical control is achieved. This dose should be continued for at least four months after the disease appears to be clinically inactive. Long-standing remissions up to 2.5 years have been reported . In an isolated report of a patient who failed oral and intralesional glucocorticoids and systemic antibiotics, application of 0.05 percent isotretinoin gel and 1 percent clindamycin gel for two months resulted in resolution of inflammation and hair growth. The outcome was maintained with the application of 0.05 percent isotretinoin gel for another eight months. The frequency of applications was not specified. The patient did not relapse for at least one year after discontinuation of treatment .
Oral zinc sulfate, oral and intralesional glucocorticoids, oral antibiotics which include fluoroquinolones, tetracyclines, dapsone, and colchicine are variably effective .
Complete scalp excision followed by split-thickness skin grafting was first reported as a successful therapeutic option in 1986 . Since then, there have been other reports that advocate the use of this treatment approach for aggressive, recalcitrant disease; in one report, the patient continued to be free of disease activity ten months post-operatively . Of note, there is a report of a case of localized dissecting cellulitis that was treated with surgical excision, which recurred one year later at the site of the surgical scar .
Selective follicular destruction has been reported to be an effective definitive therapeutic alternative in patients with treatment-refractory disease. Various modalities have been utilized for this purpose, which include X-ray radiation , 800 nm pulsed diode , long pulse Nd:YAG, and long pulse ruby lasers,  and electron beam radiation .
There are now three reports of patients with dissecting cellulitis who have been treated successfully with tumor necrosis factor-alpha blockers. A patient with disease progression despite treatment with dapsone, doxycycline, ciprofloxacin, and isotretinoin, was given infliximab at a dose of 5 mg/kg every 8 weeks for 12 months. Re-growth of hair was noted after the second infusion. One year after discontinuation of infliximab, the response was maintained with no signs of residual inflammation or relapse of the disease . In another report of a 39-year-old man who relapsed after treatment with multiple courses of antibiotics, intralesional glucocorticoids, surgical excision, and isotretinoin, it was found that adalimumab, at standard dosing of 80 mg subcutaneously (SC) for the first week, followed by 40 mg SC in the second week, and then 40 mg SC every other week thereafter, resulted in an appreciable response. At a one month follow-up visit, the patient reported that the pain and purulent discharge had stopped. At a two month follow-up visit, the patient had evidence of hair growth and at a five month follow-up visit, lesions had cleared and hair was growing normally. Adalimumab was continued . In 2010, three men, aged 27, 29, and 30 years, with treatment refractory disease were treated with standard doses of adalimumab. Clinical symptoms subsided within eight weeks of treatment in all three patients. After three months, clinical activity and subjective symptoms were reduced. However, biopsy findings during treatment demonstrated that, although the inflammatory infiltrate was reduced in two of the three patients, preexisting pathologic residual structures, such as subcutaneous sinus tracts, remained unchanged. Ultimately, when treatment with adalimumab was paused in patients after months of successful treatment, disease activity returned within four weeks and adalimumab had to be started again .
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