Cicatricial Alopecia – a Case Study

CLINICAL

Cicatricial alopecia – a case study

Siew Kim Kwa, Esha Das Gupta

Case An examination of the scalp revealed with adherent scales. This leads to A woman, 30 years of age, presented focal coin-shaped, scaly, erythematous destruction of hair follicles and permanent with patchy hair loss on her head. It lesions, some of which had coalesced balding. Initial manifestation of DLE can started 1 year ago when she developed (Figure 1). Closer examination revealed be difficult to recognise. Early referral to an itchy, slightly painful, red scaling shiny, central hypopigmentation and a dermatologist is recommended if in rash on her scalp 1 month after she absence of hair follicles, and some doubt, or if there is disease progression dyed her hair. She had been treated thickened areas of hyperpigmention and treatment failure. with antifungal and other treatments peripherally. Where there were no Alopecia areata can cause patchy unknown to her by several primary care lesions, her hair was black, thick, hair loss but does not cause scaling doctors. However, the lesion worsened, straight and long. At the periphery of the or scarring. It is characterised by and new lesions appeared. She was lesions, her hair became sparse and thin. ‘exclamation mark’ hair. referred to a dermatologist. Aside from A full examination of her face, neck, Differential diagnoses for cicatricial the devastating psychological effects body and nails revealed no other skin (scarring) alopecia include trauma, from the disfigurement and the effect lesions. burn, tinea capitis, lichen planus, scalp on her occupation as a cosmetics sales psoriasis and sarcoidosis. The history of promoter, she had no other symptoms. Question 1 hair dyeing 1 year ago might suggest a There was no significant past or family What differential diagnoses should be chemical burn, but this should not have history. She did not smoke, but her considered? progressed and no new lesions should husband smoked heavily in the house. have appeared. Question 2 Tinea capitis is associated with scaling, What investigations would be helpful in and severe lesions (kerion) can scar, but making a definitive diagnosis? it usually affects children, individuals who are immunocompromised and those in Answer 1 contact with animals. On the basis of the clinical features, Lichen planus lesions are erythematous including nummular-shaped lesions with and hyperkeratinised. They can cause central permanent scarring alopecia temporary or permanent scarring alopecia due to total loss of hair follicles, and but have characteristic white, lacy peripheral hyperpigmented adherent patterns on the buccal mucosa and nail thick scales, the most likely diagnosis is changes. discoid lupus erythematosus (DLE).1–4 Psoriatic lesions are red, scaly, DLE is the most common form of chronic hyperkeratinised and itchy. Scalp cutaneous lupus erythematosus and is psoriasis can progress to alopecia, but usually localised on the face (eg cheeks, it is associated with arthritis, plaques nose and ears). It is termed ‘generalised’ elsewhere, nail changes of pitting, and if found on the neck, upper back and subungual hyperkeratosis. Scalp lesions Figure 1. Scalp lesions with scarring alopecia 44 x 35 mm dorsum of hands. On the scalp, it causes in psoriasis usually involve the hairline. dilatation and plugging of hair follicles Scalp sarcoidosis can also mimic DLE.5

476 REPRINTED FROM AFP VOL.44, NO.7, JULY 2015 © The Royal Australian College of General practitioners 2015 CICATRICIAL ALOPECIA CLINICAL

Answer 2 Question 5 protective clothing and headwear.12 She Microscopy and culture of skin scraping How would you manage her scalp lesions? should avoid tanning salons and prolonged and hair are required to exclude exposure to fluorescent lights with high dermatophyte infections. Question 6 UV irradiation.10 She should be taught to Scalp biopsy is necessary for the What other preventive measures are recognise early malignant skin change. diagnosis in the early stages, when the appropriate for this patient? lesions are indeterminate. Histological Answer 5 findings include thickening of the Answer 3 In the management of DLE, we aim basement membrane, hyperkeratosis, DLE is an autoimmune, connective tissue to reduce disease activity and prevent follicular plugging, and perivascular and disorder that can arise alone or as part of scarring alopecia.10,12 On the basis of perifollicular lymphocytic infiltration. the disease spectrum of systemic lupus the criteria set by the Oxford Centre Early diagnosis and treatment is erythematosus (SLE).7 It was previously for Evidence-based Medicine,13 there important to prevent disfiguring alopecia, reported that 5–10% of DLE can progress are no studies with Level 1 evidence dyspigmentation and loss of self-esteem to SLE within 3 years, but it is now for cutaneous lupus erythematosus (Figure 2).6 understood to be an estimated 16.7%.7 treatment. From clinical experience, Hence, on diagnosis of DLE, full potent topical preparations from Class Case continued screening for SLE should be conducted.8 1 or 2 corticosteroid are effective Microscopy and culture results were Referral to a rheumatologist may also be for limited DLE lesions. If these fail, negative for fungus. A biopsy was not necessary. Screening includes a thorough intralesional corticosteroids can be performed as the dermatologist was history for photosensitivity, joint pain, used, but limited to small areas. Oral able to clinically diagnose limited DLE. renal problems, fits and psychosis. A systemic steroid medicines are usually physical examination should look for signs not necessary except in severe DLE Question 3 of malar rash, oral ulcer, jaundice, pallor, because of significant side effects and What further investigations must serositis and arthritis. Investigations the large dosage required. Other useful be done once a diagnosis of DLE is should include full blood examination, topical preparations that do not cause confirmed? renal and liver function tests, urinalysis, skin atrophy are calcineurin inhibitors (eg inflammatory markers (erythrocyte tacrolimus, pimecrolimus). Question 4 sedimentation rate, C-reactive protein, For extensive lesions, with or without What is the most important preventive antinuclear antibody/extractable nuclear systemic involvement, antimalarial strategy in managing DLE? antigen antibodies, double-stranded DNA treatment (eg hydroxychloroquine, and complement levels. The patient should chloroquine, quinacrine) remain the be educated on the importance of periodic primary therapy,8,10,12,14 and may delay annual screening for SLE and to seek early the onset of SLE.15 Hydroxychloroquine consultation if symptoms are suggestive causes less severe effects on the retina, of SLE. compared with chloroquine, and remains the first line antimalarial drug of choice.16 Answer 4 Patients on antimalarial treatment should Sun protection is the most important undergo baseline and periodic retinal preventive strategy as sunlight (ultraviolet screening using newer, more objective (UV) A and B) can induce and exacerbate screening tests.17 DLE lesions.9,10 Squamous cell carcinoma, Vitamin A-derived oral retinoids are as a result of chronic inflammation and effective alternatives to antimalarial absence of melanin in hypopigmented treatment, but are best avoided because lesions, is a rare complication.11 of higher side effects and teratogenicity The patient should be advised to avoid for women in child-bearing ages, unless sunbathing, outdoor work and exposure covered by effective contraception during to sunlight during the peak hours. If sun use and up to 3 months after treatment exposure is inevitable, she should apply has ceased.8 sufficient amounts (2 mg/cm2 or about Treatment of recalcitrant lesions include half a teaspoon for the face and neck) immunosuppressants (eg methotrexate, Figure 2. Scarring alopecia with post-inflammatory dyspigmentation in advanced DLE of sunscreen lotion with SPF of >50, 30 mycophenolate mofetil, azathioprine) and minutes before exposure and use sun- immunomodulators (eg dapsone).

Dyspigmentation and scarring can be Competing interests: None. 16. Wahie S, Daly AK, Cordell HJ, et al. Clinical camouflaged with cover cosmetics and Provenance and peer review: Not commissioned, and pharmacogenetic influences on response externally peer reviewed. to hydroxychloroquine in discoid lupus use of hats, scarves and wigs. erythematosus: A retrospective cohort study. J Invest Dermatol 2011;131:1981–86. Resources Answer 6 17. Marmor MF, Kellner U, Lai TY, Lyons JS, Mieler Useful websites: WF. Revised recommendations on screening for She should be screened and monitored • http://dermnetnz.org/immune/cutaneous-lupus. chloroquine and hydroxychloroquine retinopathy. periodically for vitamin D levels. If there html [Accessed 5 October 2014]. Ophthalmology 2011;118:415–22. • www.americanskin.org/resource/lupus.php 18. Piette EW, Foering KP, Chang AY, et al. Impact is a deficiency, vitamin D supplements [Accessed 5 October 2014]. of smoking in cutaneous lupus erythematosus. (400 IU/day) can be prescribed to Arch Dermatol 2012;148:317–22. counteract the lack of sun exposure.10 References 19. Dutz J, Werth VP. Cigarette smoking and Response to Antimalarials in Cutaneous Lupus 10 1. Fitzpatricks TB, Johnson RA, Klaus W, Saavedra Smoking exacerbates DLE, but AP. Color Atlas and Synopsis of Clinical Erythematosus Patients: Evolution of a Dogma. there is controversy regarding its effects Dermatology, Seventh Edition. New York: J Invest Dermatol 2011;131:1968–70. and whether it affects the response McGraw-Hill Companies, 2013. 2. Thiedke CC. Alopecia in Women. Am Fam 18,19 to treatment with antimalarials. Physician 2003;67:1007–14. Nevertheless, she should be advised to 3. Grinzi P. Hair and nails. Aust Fam Physician avoid secondary smoke exposure. Her 2011;40:476–84. 4. Walling HW, Sontheimer RD. Cutaneous husband should be strongly counselled to lupus erythematosus: Issues in diagnosis and stop smoking for their health. treatment. Am J Clin Dermatol 2009;10:365–81. 5. Tsai CF, Lee HC, Chu CY. Sarcoidal alopecia mimicking discoid lupus erythematosus: Key points Report of a case and review of the literature. • DLE appears as red scaly patches, Dematologica Sinica 2014;32:43–46. 6. Panjwani S. Early diagnosis and treatment of leaving post-inflammatory discoid lupus erythematosus. J Am Board Fam dyspigmentation and scarring. Med 2009;22:206–13. • DLE may involve hair follicles and 7. Grönhagen CM, Fored CM, Granath F, Nyberg F. Cutaneous lupus erythematosus and the lesions elsewhere (eg cheeks, nose, association with systemic lupus erythematosus: ears, neck, upper back, hands). a population-based cohort of 1088 patients in Sweden. Br J Dermatol 2011;164:1335–41. • Non-reversible scarring alopecia will 8. Okon LG, Werth VP. Cutaneous lupus develop if hair follicles are destroyed. erythematosus: diagnosis and treatment. Best • An estimated 16.7% may go on to Pract Res Clin Rheumatol 2013;27:391–404. 9. Lehmann P, Hölzle E, Kind P, Goerz G, Plewig develop SLE. Screening for SLE annually G. Experimental reproduction of skin lesions in and looking for new symptoms is lupus erythematosus by UVA and UVB radiation. recommended. J Am Acad Dermatol 1990;22:181–87. 10. Kuhn A, Ruland V, Bonsmann G. Cutaneous • Sun protection is the most important lupus erythematosus: Update of therapeutic preventive strategy as sunlight can options part 1. J Am Acad Dermatol 2011;65:e179–93. induce and exacerbate lesions. 11. Tao J, Zhang X, Guo N, et al. Squamous • Management is aimed at reducing cell carcinoma complicating discoid lupus disease activity, and this can be erythematosus in Chinese patients: Review of the literature, 1964–2010. J Am Acad Dermatol done with topical or intralesional 2012;66:695–96. corticosteroids. 12. Winkelmann RR, Kim GK, Del Rosso JQ. • Monitor vitamin D levels for deficiency Treatment of Cutaneous Lupus Erythematosus: Review and Assessment of Treatment Benefits and prescribe supplements if necessary. Based on Oxford Centre for Evidence-based • Advise on the importance of smoking Medicine Criteria. J Clin Aesthet Dermatol 2013;6:27–38. cessation. 13. Oxford Center for Evidence-Based Medicine. OCEBM Levels of evidence. Oxford: OCEBM, Authors 2014. Available at www.cebm.net/ocebm-levels- Siew Kim Kwa MBBS Malaya, MSc Medical of-evidence/ [Accessed 5 June 2015]. Demography (Univ of London), FAFPM (Academy 14. Chang AY, Piette EW, Foering KP, Tenhave TR, of Medicine of Malaysia), FRACGP, Professor and Okawa J, Werth VP. Response to antimalarial Head, Department of Family Medicine, International agents in cutaneous lupus erythematosus: Medical University, Seremban, Malaysia. A prospective analysis. Arch Dermatol [email protected] 2011;147:1261–67. Esha Das Gupta FRCP (Lond), FRCP (Ire), Head of 15. James JA, Kim-Howard XR, Bruner BF, et Division of Medicine, and Professor, Department of al. Hydroxychloroquine sulfate treatment is Internal Medicine, International Medical University, associated with later onset of systemic lupus Seremban, Malaysia erythematosus. Lupus 2007;16:401–09.