Management of Primary Cicatricial Alopecias: Options for Treatment

M.J. Harries; R.D. Sinclair; S. MacDonald-Hull; D.A. Whiting; C.E.M. Griffiths; R. Paus

Disclosures

The British Journal of Dermatology. 2008;159(1):1-22. 

In This Article

Mixed Group

Acne keloidalis nuchae (AKN) is a chronic scarring folliculitis that typically affects young black males.[287] It is characterized by the development of smooth, firm follicular papules and pustules that may coalesce into larger plaques; follicular destruction and permanent hair loss ensue. Affected areas may become secondarily infected, resulting in discharge and pain. The occipital scalp is most commonly affected; however, lesions on the vertex and crown are not uncommon.

AKN is a misnomer: there is no follicular occlusion, as seen in acne, nor is the scarring keloid like. The pathogenesis of AKN is unknown.[326] Short-necked individuals appear predisposed and mechanical irritation has been suggested as a possible trigger.[327] Ingrowing hairs are not a feature of AKN.[326]

Histopathological analysis shows perifollicular mixed infiltrate around the isthmus and sebaceous glands. Follicular epithelial thinning and lamellar fibroplasias develop with extrusion of hair shafts that elicit a granulomatous reaction. In late disease sebaceous glands are absent and marked fibrosis occurs.[326]

Treatment.

First-line treatment.

Potent topical steroid (Level of evidence = B)

Oral antibiotics PLUS topical steroids/intralesional triamcinolone acetonide (Level of evidence = E)

Twenty patients with mild (<25papules/pustules) to moderate (25–50papules/pustules) AKN were treated with clobetasol propionate 0.05% foam twice daily in a pulsed-dose regimen for 8weeks followed by betamethasone valerate 0.12% foam for a further 4weeks, in a 12-week open-label trial. Eighteen patients completed the study and all showed reduced papule count, reduced symptoms and improved investigator global assessment. The foam formulation was shown to be cosmetically acceptable which, the authors suggest, may improve patient compliance.[328] Sperling et al.[326] recommend combination treatment with a potent topical steroid (e.g. fluocinonide) combined with an oral antibiotic (e.g. tetracycline); however, no patient details were supplied. Dinehart et al.[329] reported one patient who responded to oral tetracycline. Mahe[330] proposes macrolides or cyclines as first-line treatments for AKN. ILTAC (10mgmL−1) injected every 2months showed good response in two patients.[327,329] Combination treatment of an oral tetracycline antibiotic combined with regular ILTAC injections (10mgmL−1) is recommended by Whiting.[8]

Second-line treatment.

Surgical excision (Level of evidence = B)

Carbon dioxide laser excision (Level of evidence = C)

Diode laser hair epilation (Level of evidence = E)

Surgery is recommended in patients who are resistant to medical treatment. Various surgical methods have been described but, to be successful, subfollicular destruction of the process is necessary.[331] Twenty-five patients underwent excision with primary closure (n = 24) or secondary intention healing (n = 1), with a good cosmetic outcome being seen in all. Fifteen developed small papules within the scar and five developed hypertrophic scarring.[332] Smith and Odom reported a 35% recurrence rate after excision with primary closure or skin grafting.[333] Glenn et al. reported six cases treated with excision to deep fascia followed by secondary intention healing; particularly good results were seen when an elliptical excision involving the hairline was used.[334] Dinehart et al., Califano et al. and Bennett et al. all propose deep excision with secondary intention healing as the preferred treatment for AKN.[331,335,336] Cosman and Wolff reported better cure rates and reduced recurrences with wide excision and skin grafting compared with primary closure; however, case details were not supplied.[337] Others also reported benefit with surgery.[338,339,340] Carbon dioxide laser excision was deemed better than vaporization in an eight-patient study.[341] Two patients treated with diode laser epilation achieved 90–95% clearance after four treatments.[342]

Third-line treatment.

Excision with postoperative radiotherapy (Level of evidence = E)

Isotretinoin (Level of evidence = E)

Diethylstilbestrol (Level of evidence = E)

Postoperative radiotherapy is advocated by Regan.[343] However, the well-known long-term risks of radiotherapy render it difficult to justify this approach. Isotretinoin has been reported in two cases[344,345] (again, the reservations with retinoid therapy in PCA listed above apply). Two male patients treated with diethylstilbestrol 10mg twice daily had 'satisfactory improvement' in their condition.[346]

Other.

Ciclosporin (?ineffective–Level of evidence = E)

Four patients developed AKN during ciclosporin treatment following organ transplantation. Ciclosporin-induced hypertrichosis was proposed as a possible aetiological factor in the development of lesions in these patients.[347,348]

Acne necrotica varioliformis (ANV) is an uncommon disorder characterized by erythematous papulopustules that undergo central necrosis with resulting scar formation.[8] Recurrent crops of lesions typically involve the anterior scalp but may also involve the eyebrows, nose, neck and chest. The initial oedematous lesions become umbilicated and then develop a central adherent crust, which is shed within a few weeks revealing a varioliform scar. The condition tends to occur from the third decade onwards and runs a chronic course. It is important to differentiate ANV from acne necrotica miliaris (ANM). ANM is characterized by intensely itchy follicular vesicopustules that do not scar. It is likely to represent a variant of the same process whose expression depends on individual host responses.[349]

Histopathological analysis reveals a dense perivascular and perifollicular lymphocytic infiltrate with prominent subepidermal oedema. Necrosis of individual keratinocytes is initially seen and is soon followed by confluent necrosis of the central follicle and interfollicular epidermis. The pathogenesis of ANV is unknown but suggested causes include an abnormal immune response to S.aureus, a variant of rosacea or as a result of autoinduced manipulation.[350,351]

Treatment.

First-line treatment.

Antibiotics based on microbiological culture (Level of evidence = D)

Empirical tetracycline antibiotics (Level of evidence = E)

An association between ANV and S. aureus is well documented. In 1924 Strumia demonstrated induction of ANV lesions by intradermal injection of staphylococci and streptococci isolated from a patient's lesion.[352] Clinical improvements have been reported with both culture-determined and empirical antibiotic regimens.[349,351,353] Relapses are common, therefore frequent courses to treat flares, or maintenance therapy, are often required. Some advocate measures such as doxepin, to reduce self-manipulation and trauma to the lesions.[350]

Second-line treatment.

Oral isotretinoin (Level of evidence = E)

Intralesional triamcinolone acetonide (Level of evidence = E)

Staphylococcal toxoid injections (Level of evidence = E)

Oral isotretinoin (1–2mgkg−1 daily) for 20weeks is reported to be beneficial, with most of Maibach's patients being 'symptom free without therapy' after this treatment.[354] Unfortunately no patient details were supplied. Others have also reported benefit.[351,355] ILTAC (5mgmL−1) is reported by Whiting as 'the most effective treatment for persistent lesions', although no patient details were supplied.[8] Staphylococcal toxoid injections were beneficial in one patient.[356]

Erosive pustular dermatosis (EPD) is an uncommon pustular disorder of the scalp that was first described in 1979.[357] EPD is characterized by crusted, eroded and pustular lesions, confined to the scalp, which result in scarring alopecia. It has a slowly progressive course and is usually asymptomatic. It predominantly affects elderly patients. Microbiology of early lesions is usually sterile; however, several different organisms have been isolated, probably resulting from secondary colonization;[358] the lack of response to antimicrobial therapy supports this view (see below).

Histology is nonspecific, with variable degrees of epidermal atrophy, erosions and pustule formation being present. The dermis contains a mixed chronic inflammatory infiltrate with reduced or absent hair follicles but preservation of the arrector pili muscle. The pathogenesis is unknown; however, most reports describe some form of preceding trauma to the scalp. This led Grattan et al.[359] to propose that EPD is a 'nonspecific inflammatory response to injury of aging and sun-damaged scalps'. Secondary carcinoma has been reported in one long-standing case.[360]

Treatment.

First-line treatment.

Topical corticosteroids (Level of evidence = D)

A number of case reports and two small series suggest that EPD is steroid responsive.[357,358,359,361,362,363,364,365,366,367,368,369,370,371] Rapid improvement is described with twice daily applications of potent or superpotent topical steroids. Relapses are, however, common when treatment is stopped, necessitating maintenance therapy.

Second-line treatment.

Topical immunomodulators (Level of evidence = E)

Calcipotriol cream (Level of evidence = E)

Oral zinc sulphate (Level of evidence = E)

Oral nimesulide (Level of evidence = E)

Oral corticosteroids (Level of evidence = E)

Isotretinoin (Level of evidence = E)

Topical tacrolimus 0.1% ointment, applied twice daily, has been reported in five patients with EPD and steroid-induced/actinic-induced skin atrophy.[362,364,371] All responded rapidly to treatment and improvement in skin atrophy was also observed. The advantage of avoiding steroid-induced atrophy must, however, be balanced against the theoretical risk that these agents may increase the risk of skin cancer when used on chronically sun-exposed skin.[372]

Application of 0.005% calcipotriol cream (twice daily for 2months) resulted in sustained remission at 12months follow-up in one elderly patient.[373] Oral zinc sulphate (30mg three times daily for 1week) resulted in rapid improvement in one case, with the benefit being maintained with continued treatment.[374] Others have also reported benefit with oral zinc, either as monotherapy[375] or in combination with topical corticosteroids.[376,377]

A 60-day course of oral nimesulide, an inhibitor of granulocyte respiratory burst activity, resulted in partial clearing in one patient.[358] There are mixed reports of oral isotretinoin in EPD. Some show benefit,[378,379] whereas others do not.[361,362,367]

Other.

Oral and topical antibiotics (?ineffective–Level of evidence = D)

Dapsone (?ineffective–Level of evidence = E)

Oral and topical antibiotics appear ineffective,[357,359,362,363,373,380] as does dapsone.[357,362,367]

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