PHOTO ROUNDS Somya Abubucker, MD; Bernard Cohen, MD Depigmented plaques on vulva University of Hawaii, Honolulu (Dr. Abubucker); Johns Hopkins University School of Medicine, The distinctive pattern of our young patient’s skin Baltimore, Md (Dr. Cohen) changes made the diagnosis clear. [email protected] DEPARTMENT EDITOR Richard P. Usatine, MD University of Texas Health a mother brought her 8-year-old besides herself. Her mother was concerned at San Antonio daughter to our office for evaluation of vit- that the white spots might spread to the rest The authors reported no potential iligo “down there” (FIGURE). The skin erup- of her daughter’s body, which could affect her conflict of interest relevant to this tion first appeared on her vulva a year earlier socially. article. and was intermittently pruritic. The lesions were initially smaller and red, but had since lightened in color, coalesced, and had begun ● WHAT IS YOUR DIAGNOSIS? to spread to the perianal area. The patient’s mother had received a call from her daugh- ● HOW WOULD YOU TREAT THIS ter’s teacher who observed that her daughter PATIENT? was scratching the area and might be mastur- bating in class. FIGURE The mother reported that 6 months Depigmented plaques earlier, her daughter had experienced bloody spots in her underwear accompanied by dys- in a figure-8 pattern uria. The mother brought her to the emer- gency department, where she was treated with antibiotics for a urinary tract infection. Our physical examination revealed well- circumscribed, symmetric, depigmented, confluent, crinkled, parchment-like plaques with small hemorrhagic erosions on the me- dial labia majora and minora. The lesions had spread to the perianal area with depigmenta- OF: BERNARD COHEN, MD IMAGE COURTESY tion superiorly and hypopigmentation inferi- orly, creating a figure-8 pattern. A review of systems was negative for pru- ritus, pain, dysuria, dyschezia, constipation, and vaginal discharge. The patient denied sex- ual activity, depression, or anxiety. Her mother denied behavioral changes in her daugh- ter and said that her daughter hadn’t had any one-on-one time alone with any adults PHOTO ROUNDS FRIDAY Each Friday, The Journal of Family Practice posts a new photo with a brief description and challenges you to make the diagnosis. Test your skills today! mdedge.com/jfponline MDEDGE.COM/JFPONLINE VOL 67, NO 3 | MARCH 2018 | THE JOURNAL OF FAMILY PRACTICE 171 PHOTO ROUNDS Diagnosis: der, which is most commonly vitiligo, alopecia Lichen sclerosus areata, or thyroid disease.5 In addition, 67% Based on the history and clinical findings, of patients have autoantibodies against extra- including the classic figure-8 pattern, we di- cellular matrix protein 1, and 30% have them agnosed childhood lichen sclerosus (LS) in against bullous pemphigoid antigen 180.1,8 this patient. LS is a chronic inflammatory ❚ Genetics. LS is associated with certain skin disorder that primarily affects the geni- human leukocyte antigen class II haplotypes tal mucosa. The disorder can present at any (especially DQ7) and with polymorphisms age, but is most common among postmeno- at the interleukin-1 receptor antagonist gene pausal women, with a prevalence estimated to locus.5,6,9 be as high as one in 30.1-3 A second incidence ❚ Hormones. The clear peaks of incidence peak is observed in prepubescent girls, with a during times of low estrogen, and a higher in- prevalence of one in 900.3,4 LS is less common cidence in patients with Turner syndrome or in men and boys, with a female-to-male ratio kidney disease, suggest that low estrogen may that can reach 10:1.5 The classic symptoms of play a role in the development of LS, as well.1,5,6 LS are pruritus and pain, which may be inter- While it is generally accepted that trauma mittent or persistent. may trigger LS via the Koebner phenomenon ❚ In girls, initial manifestations may be (the appearance of lesions at the site of injury), constipation, dysuria, or even behavioral there is debate as to whether microbes—espe- Up to 84% symptoms such as night fears, which can oc- cially Borrelia burgdorferi and human papillo- of cases of cur because children are less active at night mavirus (HPV)—might play a role.1,5 childhood lichen and become more aware of urinary discom- sclerosus are fort.1,2,6 Typical signs of LS are thin atrophic misdiagnosed, plaques that spare the vagina and cervix. The Diagnosis is often delayed, with an average plaques can be ivory-white, erythematous, or misdiagnosis is common delay of more violaceous. Some patients have perianal le- The average delay from symptom onset to than a year sions as well, and can display the pathogno- diagnosis of LS is 1.3 years, and up to 84% of between monic figure-8 pattern of porcelain plaques childhood LS is misdiagnosed before refer- symptom onset around the vulva and anus.5 ral.2,9 The differential diagnosis includes: and diagnosis. With more advanced disease, erosions, li- ❚ Sexual abuse. In prepubertal girls pre- chenification, and even distortion of vulvar ar- senting with genital redness, the can’t-miss chitecture may occur.2,4,7 In severe cases, labia diagnosis is sexual abuse, which occurs in resorption and clitoral phimosis may develop.5 more than 25% of children in the United Complications include secondary infection, States.10 Initial manifestations may be regres- dyspareunia, and psychosexual distress. The sion in developmental milestones, such as most worrisome sequela of LS is squamous new-onset bedwetting, or behavioral changes cell carcinoma of the vulva (SCCV), which oc- such as social withdrawal or declining aca- curs in 5% of female patients with LS.4 demic performance.11 ❚ In men and boys, LS typically involves However, physicians must be consci- the foreskin and the glans, while sparing the entious about ruling out medical etiologies perianal region.5 Scarring of the foreskin can before prematurely diagnosing abuse. Four- lead to phimosis, and patients may complain teen percent of girls with LS are incorrectly of painful erections and difficulty urinating. LS diagnosed as having been sexually abused.2 can also occur away from the genitalia in both A clinical pearl is that while LS may resemble males and females. abuse on exam, it rarely affects the hymenal structure.12 It is also important to keep in mind Autoimmune mechanisms, that the 2 entities are not incompatible, as sex- genetics, and hormones play a role ual abuse leading to LS via Koebnerization is a The exact pathogenesis of LS remains un- well-described phenomenon.12 known, but multiple factors are likely at work. ❚ Lichen planus. LP, which is also an ❚ Autoimmune mechanisms. Up to 60% immune-mediated inflammatory disorder af- of women with LS have an autoimmune disor- fecting the vulva, classically presents with the 172 THE JOURNAL OF FAMILY PRACTICE | MARCH 2018 | VOL 67, NO 3 6 Ps: pruritic, polygonal, planar, purple pap- groin creases in what is called inverse psoria- ules and plaques.4 LP is distinguished from LS sis. In addition, psoriasis tends to involve mul- by being rare in childhood, having a predilec- tiple areas, including the extensor surfaces of tion for the flexor wrists, and involving the oral the elbows and knees, the nails, and the scalp. and vaginal mucosa.4 ❚ Vitiligo can present on the genitals as ❚ Lichen simplex chronicus (LSC) is a circumscribed hypopigmented and depig- chronic, circumscribed, pruritic, eczematous mented patches that are flat. Vitiligo is as- condition that becomes lichenified with thick- ymptomatic, and the only pathology is the ened skin secondary to repeated scratching.13 change in skin color. With LS, there is licheni- Children with atopic dermatitis can develop fication, atrophy, and sclerosis.4 Vitiligo often LSC, but other children can also develop the occurs with bilateral symmetric involvement scratch-itch cycle that results in the thick- in areas of trauma including the face, neck, ened plaques of LSC. Like LS, LSC can occur scalp, elbows, wrists, hands, knees, ankles, in areas other than the genitalia, including the and feet. neck and feet.14 ❚ Allergic contact dermatitis can occur in Treatment aims to the genital area from diaper creams, soaps, and improve symptoms perfumes. Irritant contact dermatitis can oc- LS is usually diagnosed clinically (especially cur from exposure to diarrhea, bedwetting, and in children, as a biopsy is a great challenge other irritants. Contact dermatitis is less likely to perform). However, when the clinical pre- to have the classic figure-8 pattern seen in LS. sentation is unclear, a skin biopsy will dem- ❚ Psoriasis in the genital area can be con- onstrate the diagnostic findings of thinning of fused with LS. However, psoriasis favors the the epidermis, loss of rete pegs, hyperkerato- THE STRENGTH TO HEAL and get back to what I love about family medicine. Do you remember why you became a family physician? When you practice in the Army or Army Reserve, you can focus on caring for our Soldiers and their Families. You’ll practice in an environment without concerns about your patients’ ability to pay or overhead expenses. Moreover, you’ll see your efforts making a difference. To learn more, call 440-891-1800 or visit healthcare.goarmy.com/kf83. ©2010. Paid for by the United States Army. All rights reserved. MDEDGE.COM/JFPONLINE VOL 67, NO 3 | MARCH 2018 | THE JOURNAL OF FAMILY PRACTICE 173 Army ad layout.indd 1 2/20/18 11:07 AM PHOTO ROUNDS sis, and dermal fibrosis with a T-lymphocyte- daily for 8 weeks. We stressed the importance dominant inflammatory infiltrate.1,2,4,5 of genital self-examinations using a mirror to LS is a remitting and relapsing condition monitor for any concerning changes such as with no cure.