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Home , Acne, Acne conglobata, Acne keloidalis nuchae, Folliculitis decalvans, Hidradenitis, Keloid

Tyler A. Moss, DO; Thomas M. Alopecia with perifollicular Beachkofsky, MD; Samuel F. Almquist, MD; Oliver J. Wisco, DO; and pustules Michael R. Murchland, MD A.T. Still University, Our 23-year-old patient thought his was Kirksville College of Osteopathic Medicine, probably “genetic.” But that didn’t explain the painful Kirksville, Mo (Dr. Moss); Kunsan AB, Republic of pustules. Korea (Dr. Beachkofsky); Wilford Hall Medical Center, Lackland AFB, Tex (Drs. Almquist, Wisco, and Murchland) A 23-year-old African American man Physical examination revealed multiple sought care at our medical center because perifollicular papules and pustules on the [email protected] he had been losing hair over the vertex of his vertex of his scalp with interspersed patches DEPARTMENT EDITOR scalp for the past several years. He indicated of alopecia (FIGURE 1). Th ere were no lesions Richard P. Usatine, MD that his father had early-onset male patterned elsewhere on his body and his past medical University of Texas Health alopecia. As a result, he considered his hair history was otherwise unremarkable. Science Center at San Antonio loss “genetic.” However, he described waxing and waning fl ares of painful pustules associ- ● The authors reported no WHAT IS YOUR DIAGNOSIS? potential confl ict of interest ated with occasional spontaneous bleeding relevant to this article. and discharge of purulent material that oc- ● HOW WOULD YOU MANAGE curred in the same area as the hair loss. THIS PATIENT?

FIGURE 1 Alopecia with a painful twist A B PHOTOS COURTESY OF: OLIVER J. WISCO, DO

This 23-year-old patient said that he had spontaneous bleeding and discharge of purulent material in the area of his hair loss.

JFPONLINE.COM VOL 60, NO 2 | FEBRUARY 2011 | THE JOURNAL OF FAMILY PRACTICE 95 Diagnosis: men. It is considered part of the follicular oc- decalvans clusion tetrad that also includes (FD) is a highly infl am- suppurativa, conglobata, and pilonidal matory form of scarring alopecia character- . It presents as a scarring alopecia with ized by infl ammatory perifollicular papules fi rm scalp nodules that rapidly develop into and pustules. Th e term scarring alopecia re- boggy, fl uctuant, oval to linear sinuses that fers to the fact that the follicular epithelium may eventually discharge purulent material. has been replaced by , ul- In contrast to FD, dissecting scalp cel- timately resulting in permanent hair loss. lulitis lesions interconnect via sinus tract for- Th is manifests clinically as patches of skin mation so that pressure on one fl uctuant area without terminal or vellus hairs, whereas a may result in purulent discharge from perfo- nonscarring alopecia would demonstrate rations several centimeters away.5 Although preservation of the vellus hairs. Left un- both dissecting and FD are consid- treated, advancing permanent hair loss en- ered primary neutrophilic scarring alopecias, sues and may result in an end-stage pattern the presence of true sinus tract formation can of or polytrichia, where in- be a distinguishing fi nding. terspersed dilated follicular openings house ❚ CCCA is the most common form of - multiple hairs. ring alopecia among African Americans and Aff ected areas commonly include the is particularly seen among African American Folliculitis vertex and occipital scalp. Common symp- women.5 It generally presents on the scalp decalvans has toms include , itching, burning, and vertex like FD, but it is much less infl amma- been linked occasionally spontaneous bleeding or dis- tory and typically causes only mild pruritus or to scalp charge of purulent material.1 tenderness of the involved areas. colonization FD generally occurs in young and Although numerous theories have been with S aureus. middle-aged African Americans with a slight suggested, the etiology is unknown. Th e predominance in males. It accounts for 11% pathogenesis is thought to be associated with of all primary scarring alopecias.2,3 Th e etiol- premature of the inner root ogy of this infl ammatory process is not fully sheath, which can be demonstrated on bi- understood; however, scalp colonization with opsy. Also seen histologically is lymphocytic has been implicated as perifollicular infl ammation and polytrichia.6 a contributing factor.4 Other reports suggest ❚ is also a scar- patients may have an altered host immune ring alopecia. It is seen most commonly response and/or genetic predisposition for in African American men and presents as this condition.2,3 -like papules and plaques with occa- sional pustules characteristically on the oc- cipital scalp and posterior neck. In contrast to The differential includes various FD, acne keloidalis nuchae papules coalesce scarring, nonscarring alopecias and may form fi rm, hairless, protuberant Since clinical fi ndings of FD can range from keloid-like plaques that may be painful and relatively nonspecifi c mild disease at its on- cosmetically disfi guring. Th e cause of acne set to the end stage described above, a de- keloidalis nuchae is unknown. tailed patient history is needed. Th e following Shaving or cutting tight curly hair too scarring and nonscarring alopecias should short and subsequently having the new hair be considered in the diff erential diagnosis: curve back and penetrate the skin may be the dissecting cellulitis of the scalp, central cen- precipitating event. Th us, a history of close trifugal cicatricial alopecia (CCCA), acne ke- shaving should make one suspect this diag- loidalis nuchae, erosive pustular dermatosis, nosis. Histologic analysis reveals a chronic, lichen planopilaris (LPP), infl ammatory tinea predominantly lymphocytic folliculitis with capitis, and secondary . eventual follicular destruction. ❚ Dissecting cellulitis of the scalp is a ❚ Erosive pustular dermatosis is a rare distinctive, often debilitating disease com- disorder that primarily aff ects the elderly. It is monly seen in young adult African American characterized by a chronic amicrobial pustu-

96 THE JOURNAL OF FAMILY PRACTICE | FEBRUARY 2011 | VOL 60, NO 2 ALOPECIA WITH PAPULES AND PUSTULES

lar dermatosis with extensive boggy, crusted, ❚ Secondary syphilis is usually a sexu- erosive plaques on the scalp resulting in scar- ally transmitted disease, but it can also be ring alopecia. Most cases have an onset after acquired perinatally. It often presents with a the age of 40. Th erefore, age of onset may help “moth-eaten” alopecia and should be con- diff erentiate between erosive pustular der- sidered when examining patients with patchy matosis and FD. alopecia such as that seen in FD. Th ese le- Th e cause of erosive pustular dermato- sions manifest 3 to 10 weeks after the onset of sis is unknown. It is thought to be related to primary syphilis. Early in its course, the con- local trauma, such as chronic sun exposure, dition is reversible, but if it becomes chronic, occurring months to years prior to the onset the condition will cause a scarring alopecia. of lesions or as an autoimmune process.6 His- Th e presence of other stigmata, includ- tologic specimens show nonspecifi c changes ing a generalized pruritic papulosquamous including parakeratosis or hyperkeratotic eruption with involvement of the palms and scale with or erosion of the , soles, mucosal lesions ranging from superfi - while an infl ammatory infi ltrate with lympho- cial ulcers to large gray plaques, and condylo- cytes and plasma cells is found in the . mata lata, should help to diff erentiate syphilis ❚ LPP is seen more commonly in women from FD. than men, and Caucasians are more often Serologic tests such as rapid plasma re- aff ected than African Americans. It presents agin and venereal disease research labora- with , perifollicular scale, and scat- tory assays are often preferred for routine Management tered patches of scarring alopecia. Half of screening. If the index of suspicion is high, of folliculitis involved cases develop concomitant clini- confi rmatory testing with direct antibody as- decalvans cal features of . When present, says such as a microhemagglutination assay can be diffi cult, these characteristics may help distinguish it or fl uorescent treponemal antibody absorp- and long-term from FD and other scarring alopecias.6 tion test is indicated. treatment is Th e etiology of LPP is unknown, but is often necessary. thought to be similar to the presumed cause Biopsy is needed for the diagnosis of lichen planus: a T-cell−mediated autoim- Two scalp biopsies should be performed to mune response that damages basal keratino- make the diagnosis. Recommended guide- cytes.5 Histologic fi ndings include a band-like lines for sampling the scalp include perfor- mononuclear cell infi ltrate obscuring the mance of 4-mm punch biopsies extending interface between follicular epithelium and into the at 2 diff erent clinically active sites.7 dermis at the superfi cial part of the follicle One biopsy should be processed for standard with occasional interfollicular epidermal horizontal sectioning, but the second biopsy changes consistent with lichen planus. should be bisected vertically, with half sent ❚ Infl ammatory is a com- for histologic examination and the other half mon infection of the scalp that for tissue culture (fungal and bacterial). An aff ects children and adults alike. Typically, additional subsequent biopsy for direct im- it is easily distinguished from FD. However, munofl uorescence may also be considered if severe cases may result in a highly infl amma- the initial biopsies are nondiagnostic. tory pustular eruption with alopecia—with or Bacterial and fungal cultures collected without a kerion—which can make diff eren- from an intact pustule on the scalp with a tiation diffi cult. standard culture swab should also be under- In contrast to FD, the alopecia associ- taken with pustular disease. If scale is present, ated with tinea capitis is usually nonscarring, a potassium hydroxide examination can help although this depends on the extent and establish the diagnosis of a fungal etiology. depth of infection. Also, tinea capitis may present with either discrete patches or involve the entire scalp, whereas FD is usually local- , intralesional ized to the vertex or occiput (as noted earlier). are the fi rst line of Tx Correct diagnosis can be accomplished by Management of FD can be diffi cult, and long- means of light and fungal culture. term treatment is often necessary. You’ll need

JFPONLINE.COM VOL 60, NO 2 | FEBRUARY 2011 | THE JOURNAL OF FAMILY PRACTICE 97 to explain to patients that their current hair Improvement, but anticipated chronicity loss is permanent and that the goal of treat- We prescribed oral doxycycline 100 mg twice ment is to decrease infl ammation and pre- daily for our patient, as well as clobetasol vent further balding. 0.05% topical solution, to be applied to the af- After initial bacterial cultures and sen- fected area in the morning and evening. sitivities are obtained, primary treatment is We told our patient that FD is a chronic aimed at eliminating S aureus colonization. relapsing disorder and that while we could Often, this requires oral therapy, not make the condition go away completely, most commonly doxycycline 100 mg twice we could control it. We advised the patient daily5 (strength of recommendation [SOR]: C). to follow up every 2 months for the next Topical , however, may be used in 6 months, then every 6 months to ensure mild cases; options include 2% , 1% there was no progression or need to change , 1.5% , or 2% erythro- the treatment regimen. mycin applied twice daily1 (SOR: C). In recal- Th e patient’s symptoms improved after citrant cases, a common treatment regimen the fi rst 2 months. After weaning the patient includes oral rifampin 300 mg and clindamy- off doxycycline over a 6-month period, we cin 300 mg twice daily for 10 weeks4 (SOR: C). planned to transition the patient to topical Adjunctive topical and intralesional cor- clindamycin solution twice daily. ticosteroids may help reduce infl ammation In some cases, the patient can be weaned Our patient and provide symptomatic relief from itching, off oral antibiotics once the condition is con- responded well burning, and pain. Topical class I or II corti- trolled, but for most patients, continuous sys- to oral costeroids can be used twice daily, whereas temic therapy is needed. JFP doxycycline intralesional acetonide (com- 100 mg bined with topical and/or oral antibiotics) CORRESPONDENCE may be administered every 4 to 6 weeks, start- Oliver J. Wisco, Maj, USAF, MC, FS, Department of twice daily and the Air Force, Wilford Hall Medical Center, 59 MDW/ clobetasol 0.05% ing at a concentration of 10 mg/mL1 (SOR: C). SG05D/, 2200 Bergquist Drive, Suite 1, topical solution Oral corticosteroids should only be consid- Lackland AFB, TX 78236-9908; [email protected] to the affected ered for highly active and rapidly progressive area in the symptoms. morning may also be considered as a Strength of recommendation (SOR) and evening. treatment option for FD due to its antimi- A Good-quality patient-oriented evidence crobial activity and anti-infl ammatory ac- B Inconsistent or limited-quality tion directed toward metabolism. patient-oriented evidence Relapse, however, is frequent after treatment C Consensus, usual practice, opinion, disease-oriented evidence, case series withdrawal1 (SOR: C).

References 1. Otberg N, Kang H, Alzolibani AA, et al. Folliculitis decalvans. Br J Dermatol. 1999;140:328-333. Dermatol Th er. 2008;21:238-244. 5. Bolognia JL, Jorizzo JL, Rapini RP. Dermatology. 2nd ed. St. 2. Douwes KE, Landthaler M, Szeimies RM. Simultaneous occur- Louis, Mo: Mosby Elsevier; 2008. rence of folliculitis decalvans capillitii in identical twins. Br J 6. Somani N, Bergfeld WF. Cicatricial alopecia: classifi cation and Dermatol. 2000;143:195-197. histopathology. Dermatol Th er.2008;21:221-237. 3. Chandrawansa PH, Giam YC. Folliculitis decalvans—a 7. Olsen EA, Bergfeld WF, Cotsarelis G, et al. Summary of North retrospective study in a tertiary referred center, over fi ve years. American Hair Research Society (NAHRS)-sponsored work- Singapore Med J. 2003;44:84-87. shop on cicatricial alopecia, Duke University Medical Cen- 4. Powell JJ, Dawber RP, Gatter K. Folliculitis decalvans including ter, February 10 and 11, 2001. J Am Acad Dermatol. 2003;48: tufted folliculitis: clinical histological and therapeutic fi ndings. 103-110.

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98 THE JOURNAL OF FAMILY PRACTICE | FEBRUARY 2011 | VOL 60, NO 2