Tyler A. Moss, DO; Thomas M. Alopecia with perifollicular Beachkofsky, MD; Samuel F. Almquist, MD; Oliver J. Wisco, DO; papules and pustules Michael R. Murchland, MD A.T. Still University, Our 23-year-old patient thought his hair loss 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 PHOTOS COURTESY 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- Folliculitis decalvans clusion tetrad that also includes hidradenitis Folliculitis decalvans (FD) is a highly infl am- suppurativa, acne conglobata, and pilonidal matory form of scarring alopecia character- cysts. 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 has been replaced by connective tissue, ul- In contrast to FD, dissecting scalp cel- timately resulting in permanent hair loss.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 cellulitis 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 tufted folliculitis or polytrichia, where in- be a distinguishing fi nding. terspersed dilated follicular openings house ❚ CCCA is the most common form of scar- 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 pain, 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 desquamation 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 Staphylococcus aureus has been implicated as perifollicular infl ammation and polytrichia.6 a contributing factor.4 Other reports suggest ❚ Acne keloidalis nuchae 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 keloid-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 syphilis. 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 atrophy or erosion of the epidermis, 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 dermis. 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 erythema, 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 lichen planus. 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 fat 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 tinea capitis is a com- for histologic examination and the other half mon dermatophyte infection of the scalp that for tissue culture (fungal and bacterial). An aff ects children and adults alike.