Folliculitis and Other Follicular Disorders




Abstract


This chapter reviews both superficial and deep forms of folliculitis as well as disorders of follicular keratinization and the follicular occlusion tetrad (acne conglobata, hidradenitis suppurativa, dissecting cellulitis, and pilonidal sinus). Examples of superficial folliculitis include those due to Staphylococcus aureus or Pseudomonas aeruginosa infections, whereas acne keloidalis and pseudofolliculitis barbae are examples of deep forms. The disorders of follicular keratinization represent a varied group of dermatoses that have in common prominent keratotic follicular plugs. Some of these disorders are reviewed in other chapters, e.g. lichen planopilaris, pityriasis rubra pilaris and keratosis pilaris, while others are discussed here, e.g. lichen spinulosus and erythromelanosis follicularis faciei. Lastly, there are inflammatory disorders that can have a follicular predilection such as GVHD or, in darkly pigmented skin, papular eczema and pityriasis rosea.




Keywords

folliculitis, eosinophilic folliculitis, keratosis pilaris atrophicans, lichen spinulosus, pseudofolliculitis barbae, acne keloidalis, follicular occlusion tetrad, hidradenitis suppurativa

 




Introduction


This chapter will review both superficial and deep forms of folliculitis as well as disorders of follicular keratinization and the follicular occlusion tetrad (acne conglobata, hidradenitis suppurativa, dissecting cellulitis, and pilonidal sinus). Examples of superficial folliculitis include those due to Staphylococcus aureus or Pseudomonas aeruginosa infections, whereas acne keloidalis and pseudofolliculitis barbae are examples of deep forms. The disorders of follicular keratinization represent a varied group of dermatoses that have in common prominent keratotic follicular plugs. Some of these disorders are reviewed in other chapters, e.g. lichen planopilaris, pityriasis rubra pilaris (PRP) and keratosis pilaris, while others are discussed here, e.g. lichen spinulosus and erythromelanosis follicularis faciei. Lastly, there are inflammatory disorders that can have a follicular predilection such as GVHD or, in darkly pigmented skin, papular eczema and pityriasis rosea.




Superficial Folliculitis




Synonym


▪ Impetigo of Bockhart



Key features





  • Follicular pustules, often arising on an erythematous base



  • Favors areas with terminal hairs, such as the scalp and beard, as well as the upper trunk, buttocks, and lower extremities



  • Bacterial culture of pustular contents most commonly reveals normal flora, followed by Staphylococcus aureus



  • Treatment depends on the particular type of folliculitis




Introduction


Folliculitis is a very common disorder characterized by follicular pustules. It can be secondary to a wide range of infectious agents, or be induced by irritants or drugs ( Fig. 38.1 ). Microbial cultures may fail to identify a pathogen, but, of the infectious etiologies, Staphylococcus aureus is the most common.




Fig. 38.1


Clinical approach to superficial folliculitis.

Edematous lesions of folliculitis are most suggestive of eosinophilic folliculitis, Demodex folliculitis, and Pseudomonas “hot tub” folliculitis. See Table 38.1 for medications that can cause drug-induced folliculitis.

Courtesy, Jean L Bolognia, MD.


Clinical features


Follicular pustules are usually accompanied by an erythematous rim ( Fig. 38.2 ). If no pustules are present, follicular-based erythematous papules or a superimposed collarette of scale serve as clues to the diagnosis. Lesions may be pruritic or even tender or painful. Occasionally, when there is more widespread involvement, patients will complain of generalized pruritus .




Fig. 38.2


Folliculitis (culture-negative).

Follicular pustules with an erythematous rim are present on the back of an adolescent. The differential diagnosis is primarily folliculitis due to Staphylococcus aureus and acne.

Courtesy, Julie V Schaffer, MD.


Folliculitis occurs most commonly in the head and neck region (especially the scalp and beard area), upper trunk, buttocks and legs, as well as in the axillae and groin ( Fig. 38.3 ). It favors areas that are occluded and/or have terminal hairs. In addition, shaving, especially against the direction of hair growth, can predispose to folliculitis.




Fig. 38.3


Folliculitis of the axilla and beard area due to Staphylococcus aureus .

A Numerous follicular pustules with an erythematous rim emanating from the axillary vault. B Discrete papulopustules are seen posteriorly while centrally, there is deeper involvement with plaque formation (sycosis barbae).

A, Courtesy, Kalman Watsky, MD.


Pathology


A perifollicular infiltrate of lymphocytes, neutrophils, and macrophages is seen, with extension of the neutrophils into the follicular epithelium and follicular canal aggregating variably to form an abscess. Late-stage changes include follicular rupture and a granulomatous response. Tissue Gram stain may reveal pathogenic organisms. Occasionally, cytopathic changes due to herpes simplex viral infection are seen within the follicular epithelium (see Ch. 80 ) or multiple Demodex organisms are present within the follicular canal in demodicosis (see Fig. 38.5C ), leading to the diagnosis of other forms of folliculitis.


Differential diagnosis


In addition to the various forms of folliculitis listed in Table 38.1 , the differential diagnosis also includes acne vulgaris, pseudofolliculitis barbae and, occasionally, rosacea. Microscopic examination of follicular contents may provide a clue to the type of folliculitis as can microbial cultures and histologic examination. In patients who are confined to bed, folliculitis may develop predominantly on the posterior trunk and thus needs to be distinguished from other disorders exacerbated by occlusion including Grover disease, miliaria rubra, and cutaneous candidiasis.



Table 38.1

Less common specific forms of folliculitis.

DFA, direct immunofluorescence assay; EGFR, epidermal growth factor receptor; MEK, mitogen activated protein kinase (MAPK)/extracellular signal regulated (Erk) kinase. PCR, polymerase chain reaction.














































































LESS COMMON SPECIFIC FORMS OF FOLLICULITIS
Etiology/risk factors Clinical features Therapy
Irritant folliculitis
Usually occurs following the application of topical medications (e.g. tar preparations), ointments
Exacerbated by application in a direction opposite that of hair growth



  • Follicular pustules in the sites of application or exposure to irritant



  • Favors areas with terminal hairs




  • Discontinue inciting topical agent



  • Apply medications in the same direction as hair growth



  • Topical mid-potency corticosteroid lotion

Gram-negative folliculitis
Due primarily to Klebsiella, Escherichia coli, Enterobacter and Proteus spp.
Usually seen in patients with acne vulgaris receiving long-term antibiotic therapy



  • Pustules in the facial T-zone and in a perinasal distribution



  • Adult men with oily skin




  • Topical: gentamicin, benzoyl peroxide



  • Systemic: quinolones (e.g. ciprofloxacin)



  • If severe or recurrent: isotretinoin, 0.5–1 mg/kg/day for up to 16 weeks

Hot tub folliculitis due to Pseudomonas aeruginosa in the setting of use of a hot tub/whirlpool 12–48 hours prior to onset


  • Edematous, pink to red follicular papules and pustules, primarily on the trunk ( Fig. 38.4 )



  • Often pruritic and resolves spontaneously in immunocompetent hosts



  • May be serious illness in immunocompromised hosts




  • Generally self-limited; can use antibacterial soap



  • If severe or immunocompromised host: ciprofloxacin, 500 mg po twice daily for 7–14 days



  • Water in hot tub, whirlpool or swimming pool must be maintained with chlorine (0.4–1.0 ppm, pH 7.2–7.4) and changed every 6–8 weeks to lower organic carbon level (hot tubs, whirlpools)

Dermatophyte folliculitis
Tinea barbae usually due to Trichophyton mentagrophytes or T. verrucosum
Classically occurs in male farm workers



  • Inflammatory papules and pustules in the beard area more often than the mustache, with crusting



  • Loosened hairs can be removed without pain




  • Topical antifungals (may be ineffective)



  • Terbinafine, 250 mg/day po for 2–3 weeks



  • Micronized or ultramicronized griseofulvin, 500–1000 mg/day or 500–750 mg/day po, respectively, for 4–6 weeks



  • Itraconazole, 200 mg po twice daily for 1 week per month, for 2 pulses

Majocchi granuloma, usually due to T. rubrum , characteristically develops in women who shave their legs
Additional risk factors include occlusion, immunosuppression, and use of potent topical corticosteroids



  • Follicular pustules and follicular papules or nodules ( Fig. 38.5A ), most often located on the lower legs



  • Pustules at edges of tinea cruris or corporis




  • Oral antifungal medications recommended, with treatment regimens similar to tinea barbae (see above)



  • Immunosuppressed patients may need a longer course of therapy

Malassezia (Pityrosporum) folliculitis
Typically occurs in young adults, with aggravating factors including warm weather, occlusion and excessive sebum production
May also develop in association with antibiotic therapy (especially tetracyclines) or iatrogenic immunosuppression (e.g. organ transplant recipients)



  • Pruritic follicular papules and occasionally pustules, primarily on the back, chest and shoulders



  • Central white–yellow color often represents compact keratin rather than pus



  • A KOH preparation of follicular contents reveals abundant yeast forms




  • Topical: antifungals, selenium sulfide shampoo, 50% propylene glycol in water



  • Systemic: fluconazole 100–200 mg/day for 3 weeks or 200–300 mg once weekly for 1–2 months; itraconazole 200 mg/day for 1–3 weeks

Candida folliculitis
Primarily in diabetics


  • Pruritic satellite pustules surrounding areas of intertriginous candidiasis



  • Facial lesions may mimic tinea barbae or acne rosacea




  • Prevent skin-to-skin contact in intertriginous areas



  • If possible, discontinue antibiotics and/or corticosteroid therapy



  • Mild to moderate disease: topical antifungals



  • Recalcitrant or severe disease: fluconazole 100 mg daily for 1 week then every other day for 1 month

Herpes simplex folliculitis
Herpetic sycosis is usually seen in men with a history of recurrent localized facial herpes simplex infection who shave with a blade razor
Widespread or unusual presentations of herpes simplex folliculitis may be seen in HIV-infected or otherwise immunosuppressed individuals



  • Rapid development of individual and grouped follicular pustules and vesicles on an erythematous base in the beard area



  • A Tzanck smear or biopsy reveals multinucleated giant cells



  • Positive PCR or DFA




  • Acyclovir 200 mg po 5 times per day for 5–10 days



  • Famciclovir 500 mg po three times per day for 5–10 days



  • Valacyclovir 500 mg po three times per day for 5–10 days

Demodex folliculitis (demodicosis)
May be associated with immune suppression


  • Erythematous follicular papules and pustules on the face, especially the nose, and neck; may have a background of diffuse erythema ( Fig. 38.5B )



  • Skin scrapings reveal numerous Demodex mites ( Fig. 38.5D )




  • Topical: 1% ivermectin cream, 5% permethrin cream



  • Systemic: single dose of ivermectin 200 mcg/kg po

Drug-induced folliculitis (acneiform eruption)
Most common in acne-prone patients and age groups
Corticosteroids, androgenic hormones, EGFR inhibitors, MEK inhibitors, sirolimus, iodides, bromides, lithium, isoniazid, and anticonvulsants
Can develop within 2 weeks of starting the offending agent, but the risk is often proportional to the dose and duration of therapy



  • Acute eruption of monomorphic erythematous follicular papules and pustules on the trunk, shoulders and upper arms (see Figs 21.19B & 36.13 )



  • Multiple papulopustules of the face and scalp, sometimes admixed with scale-crust (see Figs 21.19B & 36.15 )



  • Unlike acne vulgaris, comedones are rarely present (with the exception of androgens)




  • Discontinue the offending drug, if possible



  • Topical: benzoyl peroxide, clindamycin, erythromycin, retinoids



  • Systemic: tetracycline, doxycycline, minocycline

Necrotizing infundibular crystalline folliculitis
Yeasts and Gram-positive bacteria in affected follicles


  • Waxy papules that favor the forehead, neck, and back



  • Birefringent, filamentous, crystalline deposits within follicular ostia




  • Topical or systemic antimycotics

Actinic folliculitis
Seasonal
Follows initial summer sun exposure



  • Development of follicular pustules on the upper trunk and arms 24–30 hours after first sun exposure of the summer



  • Lesions spare the face and are not pruritic




  • Sun avoidance; sunscreens will not prevent lesion formation



  • Topical mid-potency corticosteroids



  • Isotretinoin 0.35–0.5 mg/kg/day for 3–6 months if severe




Fig. 38.4


Pseudomonas “hot tub” folliculitis.

Edematous follicular papules on the flank that began to develop 2–3 days following the use of a hot tub. The number and size can vary.

Courtesy, Kalman Watsky, MD.



Fig. 38.5


Folliculitis – fungal and ectoparasitic.

A Firm follicular papules of dermatophyte folliculitis (Majocchi granuloma) in the setting of extensive tinea corporis. B Numerous papules and papulopustules of Demodex folliculitis on the face and neck; note the edematous nature of several of the lesions. C Histopathologic features of Demodex folliculitis with several organisms within the follicular ostium. D Microscopic findings of follicular contents obtained via scraping of Demodex folliculitis.

B, Courtesy, Angela Hernández-Martin; C, Courtesy, Lorenzo Cerroni, MD.


Treatment


The treatment chosen depends upon the culture results and severity (see Chs 74 & 127 ). For patients whose cultures fail to demonstrate a pathogenic microbe, the treatments, as in acne, include topical benzoyl peroxide, topical antibiotics (e.g. clindamycin), and oral antibiotics (e.g. tetracycline, doxycycline), with the anti-inflammatory properties of these antibiotics probably playing a role. This should be combined with measures to reduce overhydration of the skin, occlusion, and irritation.


Eosinophilic Folliculitis


Three major forms of eosinophilic folliculitis have been described – eosinophilic pustular folliculitis (Ofuji disease), immunosuppression (or HIV)-associated eosinophilic pustular folliculitis, and eosinophilic pustular folliculitis of infancy.


Eosinophilic pustular folliculitis




Synonym


▪ Ofuji disease



Key features





  • Follicular papulopustules as well as erythematous patches and plaques with superimposed coalescent pustules; central clearing leads to figurate lesions



  • Most commonly involves the face, followed by the upper extremities and trunk



  • Moderate to severe pruritus is a frequent symptom



  • Recurrent crops of lesions that involute over 7–10 days



  • Peripheral blood eosinophilia is also seen



  • Histologically, eosinophils predominate around and within the follicular epithelium




Introduction


Eosinophilic pustular folliculitis is characterized by recurrent episodes of eruptive, intensely pruritic, follicular papulopustules. It typically affects “acne-prone” areas of the body such as the face, back, and extensor surface of the arms . To date, this disorder has not been associated with systemic disease.


History


In 1965, Ise and Ofuji were the first to report a patient who had repeated episodes of follicular pustules on the face and back with peripheral blood eosinophilia. Five years later, Ofuji et al. described three additional patients and proposed the name “eosinophilic pustular folliculitis” for this new clinical entity. Of note, this is not to be confused with papuloerythroderma of Ofuji (see Ch. 25 ).


Epidemiology


Eosinophilic pustular folliculitis is a relatively rare entity, with most of the cases reported from Japan. The majority of patients are adults and the average age of onset is 30 years. The ratio of men to women is ~5 : 1 amongst Japanese patients. There have been occasional reports of eosinophilic pustular folliculitis in children, often with clinical features similar to the infantile form (see below).


Pathogenesis


The etiology and pathogenesis of eosinophilic pustular folliculitis is unknown. Proposed mechanisms include hypersensitivity reactions to various antigenic stimuli (e.g. infectious agents, medications) and immune dysfunction .


Clinical features


Recurrent crops of intensely pruritic, grouped, follicular pustules and papulopustules develop in a somewhat explosive fashion. In addition, there may be erythematous patches and plaques with superimposed coalescent pustules; central clearing and centrifugal extension produce annular and figurate lesions. Typically, areas with “sebaceous” follicles, such as the face, back, and extensor surface of the arms, are affected. However, the digits, palms and soles may be involved, proving that not all lesions are follicular. Lesions last ~7–10 days and tend to relapse every 3–4 weeks. The patients have no accompanying systemic symptoms.


Pathology


Involved follicles may show spongiosis, with exocytosis of lymphocytes and eosinophils into the follicular epithelium. These findings can extend from the sebaceous gland and its duct up to and including the infundibular zone. A dermal infiltrate of lymphocytes and eosinophils may also be present. Micropustular aggregation develops, followed by the hallmark finding of infundibular eosinophilic pustules . Secondary follicular mucinosis is sometimes seen.


Differential diagnosis


Clinically, some of the lesions may resemble other forms of superficial folliculitis (see previous section). However, the explosive crops, annular plaques with central clearing, intense pruritus, peripheral blood eosinophilia, and histologic findings typically enable differentiation from clinically similar entities. Rarely, early eosinophilic pustular folliculitis may have a symmetric, butterfly rash-like presentation that resembles acute cutaneous lupus erythematosus.


Treatment


To date, potential treatments are based upon case reports or small series. Topical corticosteroids, tacrolimus ointment, and oral antihistamines can be prescribed for the associated pruritus. First-line treatment for eosinophilic pustular folliculitis is oral indomethacin (50 mg/day). Second-line therapies include UVB phototherapy, oral minocycline (100 mg twice daily), oral dapsone (100 to 200 mg/day for ≥2 weeks), systemic corticosteroids, and colchicine (0.6 mg twice daily). Cyclospor­ine may be considered in patients with refractory disease.


Immunosuppression-associated eosinophilic pustular folliculitis




Synonyms


▪ AIDS-associated eosinophilic folliculitis ▪ HIV-associated eosinophilic folliculitis ▪ Immunosuppression-associated eosinophilic folliculitis



Key features





  • Markedly pruritic follicular papules, urticarial and/or pustular, that favor the face, scalp, and upper trunk



  • Occurs in the setting of immunosuppression, including from HIV infection and following allogeneic hematopoietic stem cell transplantation



  • In HIV-infected patients, the CD4 count is usually <200 cells/mm 3 ; it may also be a manifestation of IRIS (immune reconstitution inflammatory syndrome)



  • Histologically, lesions are characterized by spongiosis and exocytosis of eosinophils and lymphocytes into the follicular epithelium




Introduction and history


A pruritic papular eruption with histologic features similar to eosinophilic pustular folliculitis (Ofuji disease) was originally described in HIV-infected patients, many of whom fulfilled the criteria for AIDS. Eosinophilic folliculitis has subsequently been observed in patients with lymphoma, chronic lymphocytic leukemia, acute myelogenous leukemia and other myeloproliferative disorders, as well as in those who have undergone allogeneic hematopoietic stem cell transplantation; hence, the use of the term immunosuppression-associated eosinophilic pustular folliculitis . However, in contrast to Ofuji disease, there are neither large coalescent pustules nor figurate lesions and the individual lesions are more persistent.


Epidemiology


Although the early descriptions of HIV-associated eosinophilic folliculitis were in men, it can also occur in women . Its development correlates with a low CD4 count (in particular <200/mm 3 ), and clinical improvement has been observed as the CD4 count rises due to antire­troviral therapy (ART). However, there are instances where eosinophilic folliculitis develops within 3 to 6 months of initiating ART, implying an association with the immune reconstitution inflammatory syndrome (IRIS; see Ch. 78 ).


Pathogenesis


Little is known about the exact pathogenesis of immunosuppression-associated eosinophilic folliculitis. However, as in other eosinophilic diseases, a Th2 immune response has been implicated. In patients with AIDS, elevated levels of interleukin (IL)-4, IL-5, RANTES (CCL5), and eotaxin (CCL11) mRNA were detected within lesional skin (see Ch. 25 ) . A more recent study found elevated serum levels of three additional chemokines, CCL17, CCL26 (eotaxin-3) and CCL27, in patients with HIV-associated eosinophilic folliculitis .


Clinical features


Immunosuppression-associated eosinophilic folliculitis is characterized by a chronic, pruritic, follicular papular eruption of the face, scalp, and upper trunk ( Fig. 38.6A ). The papules are often slightly edematous and pustules may or may not be present ( Fig. 38.6B ). The associated pruritus is often intense. Some patients may also have lymphopenia.




Fig. 38.6


Immunosuppression-associated eosinophilic folliculitis.

A Multiple follicular papules on the anterior trunk in a patient who had received an allogeneic hematopoietic stem cell transplant. Note the edematous nature of some of the lesions. Very few pustules were present. B In some patients, multiple follicular pustules are seen. C A dilated hair follicle with an intrafollicular collection of eosinophils; a few eosinophils are also present within the follicular epithelium.

A, Courtesy, Dennis Cooper, MD; B,C, Courtesy, Luis Requena, MD.


Pathology


The histologic features are similar to those of Ofuji disease (see above; Fig. 38.6C ) .


Differential diagnosis


The differential diagnosis includes more common causes of folliculitis (see above) as well as the entities outlined in Table 38.1 , especially Demodex folliculitis. Microscopic examination of follicular contents as well as performance of a biopsy may be necessary to establish the diagnosis (see Fig. 38.1 ). Additional disorders to consider, especially when there are no pustules and it is uncertain if lesions are follicular-based, are HIV-associated pruritic papular eruption , papular dermatitis, or arthropod bites and other causes of a “dermal hypersensitivity reaction”. The possibility of septic emboli due to an opportunistic infection depends upon the clinical setting. Necrotizing eosinophilic folliculitis is said to be distinguished by its association with atopy and the presence of nodules and ulceration, with evidence of follicular necrosis and eosinophilic vasculitis histologically.


Treatment


For HIV-infected patients, treatment of the underlying viral infection, with a resultant rise in the CD4 cell count, may lead to a resolution of lesions. In patients with IRIS-associated exacerbations, the goal is improvement despite continuation of ART as the IRIS-related disease activity gradually subsides. A need for temporary interruption of ART would be unlikely if eosinophilic folliculitis were the primary manifestation of IRIS.


For all patients, oral and topical antipruritics in combination with topical corticosteroids can be tried, but if they prove inadequate, UVB phototherapy may be required. Additional therapies include topical tacrolimus, topical permethrin, oral itraconazole (200–400 mg daily), oral metronidazole (250 mg three times daily), oral antibiotics, isotretinoin (0.5–1 mg/kg/day × 1–4 weeks, then taper), and interferon (β and γ) .


Eosinophilic pustular folliculitis of infancy




Synonyms


▪ Eosinophilic pustular folliculitis in infancy ▪ Infantile eosinophilic pustular folliculitis ▪ Neonatal eosinophilic pustular folliculitis ▪ Eosinophilic pustular folliculitis in childhood ▪ Eosinophilic pustulosis (of the scalp) in childhood



Key features





  • Unusual, self-limiting disorder that typically appears during early infancy



  • Multiple, pruritic, follicular pustules and vesiculopustules on an erythematous base



  • Seen primarily on the scalp and brow region



  • Secondary crusting is common



  • Histologically, there is a dense perifollicular infiltrate of eosinophils and lymphocytes, which may spare the sebaceous glands and outer root sheath




Introduction


Eosinophilic pustular folliculitis of infancy is a rare, self-limiting disorder of unknown etiology that occurs during infancy (see Ch. 34 ). It differs from eosinophilic pustular folliculitis in adults by primarily involving the scalp, frequently developing secondary crusting, and a lack of an annular configuration.


History


In 1984, Lucky et al. first described five infants with this disorder. To date, one HIV-related case has been reported in an infant, with an onset at 3 months of age .


Epidemiology


In a review of 61 cases of eosinophilic pustular folliculitis of infancy, disease onset was prior to age 14 months in 95% of patients . The disorder is more common in boys than girls with a ratio of 4 : 1.


Pathogenesis


The etiology of this self-limiting disorder is unknown, and the pustules are sterile. The eosinophil clearly plays a role in this disease, and the regulation of its function is reviewed in Chapter 25 . Of note, some authors have proposed that eosinophilic pustular folliculitis of infancy is better characterized as a clinicopathologic reaction pattern (with heterogeneous underlying causes such as arthropod bites or dermatophytosis) as opposed to a distinct inflammatory dermatosis.


Clinical features


Pruritic, follicular-based pustules and vesiculopustules with an erythematous rim occur primarily on the scalp. There may be involvement of the neck, face, and trunk and, occasionally, lesions appear on the extremities. The pustules often have an erythematous base and develop secondary crusting. A cyclical course, with recurrences at intervals ranging from 1 to 12 weeks, is characteristic. The majority of children experience resolution by 3 years of age.


Pathology


Typically, eosinophilic spongiosis as well as clusters of eosinophils are seen in the hair follicle, especially within the infundibular region. There are also variable numbers of neutrophils. A perifollicular inflammatory infiltrate contains eosinophils, neutrophils, lymphocytes, and histiocytes.


Differential diagnosis


The differential diagnosis includes erythema toxicum neonatorum, transient neonatal pustular melanosis, acropustulosis of infancy, scabies (which can involve the scalp in infants), Langerhans cell histiocytosis, and the following two rare disorders: papulopustular eruption of hyper-IgE syndrome and vesiculopustular eruption of transient myeloproliferative disorder. Erythema toxicum neonatorum has almost identical histologic features, but its onset is within the first 48 hours of life and the eruption, which has a more widespread distribution pattern, usually resolves within 1 week (see Ch. 34 ). This natural history easily separates the two entities.


Transient neonatal pustular melanosis occurs in 4–5% of infants with darkly pigmented skin. Lesions are present at the time of birth and favor the face, neck, and shins. Neutrophils are the predominant cells. The lesions of acropustulosis of infancy occur more frequently in male infants with darkly pigmented skin, and they are seen most often on the hands and feet and less often on the scalp, proximal extremities, and trunk. The disorder may occur during the neonatal period, but usually appears at 3–6 months of age. Lesions last 7–10 days and crops occur for months to years, usually resolving completely by the age of 3 years. Given that eosinophilic pustular folliculitis of infancy occasionally involves the extremities and has a similar natural history of cropping, some authors have suggested a relationship between the two entities.


Langerhans cell histiocytosis is a rare disorder that often has cutaneous involvement when it develops during infancy. Papules, pustules, vesicles, and crusts are seen, especially on the scalp and in flexural areas. Histologic examination demonstrates the presence of S100 + , CD1a + Langerhans cells.


Treatment


Parents should be reassured regarding the benign, self-limited nature of the disorder. Topical corticosteroids and oral antihistamines can help to relieve pruritus during flares of the disease.


Disseminate and Recurrent Infundibulofolliculitis




Synonyms


▪ Hitch and Lund disease ▪ Recurrent disseminated infundibulofolliculitis



Key features





  • Seen primarily in adults with darkly pigmented skin



  • Hundreds of uniform, skin-colored papules that have been likened to “goose bumps”



  • Favored sites of involvement are the trunk, neck, and upper extremities



  • May last for months to years and can be pruritic



  • Histologically, there is mild inflammation of the follicular infundibulum


Disseminate and recurrent infundibulofolliculitis was first described by Hitch and Lund in 1968 . Most affected individuals have darkly pigmented skin. Although the lesions may resemble papular eczema, many of the patients do not have a history of atopy.


The disease is characterized by numerous 1–2 mm, pruritic, skin-colored papules, often pierced by a hair, forming large areas of what looks like “goose bumps”. The trunk is involved most often, followed by the neck, buttocks, and arms. The lesions may last for weeks, months, or years.


Although the disorder most closely resembles papular eczema (and some authors do not distinguish between the two entities), other diseases that may be considered in the differential diagnosis are lichen nitidus and folliculitis (see above), but the follicular pattern and the lack of erythema or pustules exclude these two entities, respectively. The lack of keratotic plugs or spines within the hair follicles distinguishes this disorder from keratosis pilaris, PRP, lichen planopilaris, and the disorders discussed in the next section (e.g. phrynoderma ). Careful examination of the primary lesions excludes the diagnosis of Darier disease.


Histologically, the principal findings are in the infundibular portion of the hair follicle. There is perifollicular edema and a slight infiltration of lymphocytes with occasional neutrophils .


Topical corticosteroids are sometimes effective, and lactic acid preparations (12%) or 20–40% urea may be helpful. If topical medications prove unsuccessful, PUVA may be tried (thrice weekly for 3 weeks, followed by maintenance therapy twice a month). Vitamin A (50 000 IU twice daily) and isotretinoin (0.5 mg/kg/day for 16 weeks) have also been tried.




Disorders of Follicular Keratinization


Although this group of dermatoses has a wide spectrum of clinical presentations, all are characterized by a prominent plug of keratin within the follicular orifice. For most of these entities, the cause of the abnormal keratinization is unknown. The dermatoses are usually distinguished on the basis of the size, extent and distribution of the keratotic lesions, as well as the presence or absence of perifollicular erythema and associated scarring. Disorders in this group that are discussed in other chapters include keratosis pilaris ( Ch. 12 ), PRP ( Ch. 9 ), and lichen planopilaris ( Ch. 11 ).


Erythromelanosis Follicularis Faciei




Synonym


▪ Erythromelanosis follicularis faciei et colli



Key features





  • An uncommon disorder that involves the lateral cheeks and sometimes the lateral aspects of the neck



  • Multiple pinpoint follicular papules are superimposed on a red–brown patch; the papules often appear relatively hypopigmented



  • May be accompanied by keratosis pilaris




Introduction


Erythromelanosis follicularis faciei was first described in 1960 by Kitamura and associates in Japanese men. Six years later, the first patient was described in the English literature by Mishima and Rudner . It can begin during the second decade of life and is characterized by background erythema and hyperpigmentation as well as tiny follicular papules.


Epidemiology


Although the disorder was initially reported in Japanese men, it is now known to affect all races and both sexes . However, it does favor people of Asian ancestry.


Pathogenesis


The pathogenesis is unknown.


Clinical features


On the lateral aspect of the cheeks, and sometimes the lateral aspects of the neck, there is a background of red–brown-colored skin. The latter is due to a combination of vasodilation and hyperpigmentation. However, in skin type I patients, there may be only erythema, leading to significant overlap with keratosis pilaris rubra. Numerous pinhead-sized follicular papules are present within the involved areas, and they appear relatively hypopigmented. Patients may also have keratosis pilaris of the upper outer arms, with a small rim of erythema surrounding the follicular keratotic plug (see Ch. 12 ).


Pathology


There is slight follicular hyperkeratosis with increased epidermal pigmentation. Diameters of the hair shafts and outer root sheaths are decreased, as is the thickness of the inner root sheath. The adnexa are often surrounded by a lymphocytic infiltrate. Both the thickness and the compactness of the horny layer are usually increased . By quantitative histopathology, an increase in the percent area of superficial blood vessels was observed which correlated with visual grading of the associated erythema .


Differential diagnosis


The differential diagnosis of erythromelanosis follicularis faciei includes primarily keratosis pilaris rubra and a combination of melasma plus telangiectatic erythema from photodamage. However, the latter combination lacks tiny follicular papules, while keratosis pilaris rubra of the cheeks lacks hyperpigmentation and favors children (see Ch. 12 ).


Poikiloderma of Civatte is another entity that is sometimes confused with erythromelanosis follicularis faciei et colli. The former involves the anterolateral aspects of the neck with obvious sparing of the mid-submental region. The areas of erythema are a reflection of multiple interfollicular telangiectasias. Because the telangiectasias spare the immediate thin rim of skin around each follicle, these areas appear relatively hypopigmented. Occasionally, poikiloderma of Civatte extends onto the mandibular region.


Less often, two disorders within the spectrum of keratosis pilaris atrophicans, ulerythema ophryogenes and atrophoderma vermiculatum, may be considered ( Table 38.2 ). Although ulerythema ophryogenes is characterized by small follicular papules, they are erythematous, usually appear during early childhood, and favor the eyebrows ( Fig. 38.7 ). In addition, there is associated follicular atrophy and scarring alopecia of the lateral eyebrows. Atrophoderma vermiculatum affects the cheeks but presents with honeycomb scarring or a worm-eaten appearance (see Ch. 99 ).


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Sep 15, 2019 | Posted by in Dermatology | Comments Off on Folliculitis and Other Follicular Disorders
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