Sarcoidal Alopecia Mimicking Discoid Lupus Erythematosus Biology Essay

Dermatologica Sinica

Manuscript Draft

Manuscript Number: DSI-D-12-00070

literature

Article Type: Case report

Keywords: Sarcoidal alopecia; discoid lupus erythematosus

Abstract: Sarcoidal alopecia is a subtype of plaque-form cutaneous sarcoidosis that may resemble

discoid lupus erythematosus (DLE). The clinical appearance of the two lesions is similar, thereby

leading to diagnostic confusion. Because of the systemic involvement and progressive nature of

sarcoidosis, it is important to differentiate sarcoidal alopecia from DLE so that proper treatment can be

initiated, thereby avoiding the potential long-term sequelae. We herein present a case of Taiwanese

woman with scalp sarcoidal alopecia mimicking DLE.

*Manuscript without author details

Sarcoidal alopecia mimicking discoid lupus erythematosus: Report of a case and review of the

literature

Chia-Fen Tsai, M.D., Hsing-Chuan Lee, M.D., Chia-Yu Chu, M.D., Ph.D.

Abstract

Sarcoidal alopecia is a subtype of plaque-form cutaneous sarcoidosis that may resemble discoid lupus

erythematosus (DLE). The clinical appearance of the two lesions is similar, thereby leading to diagnostic

confusion. Because of the systemic involvement and progressive nature of sarcoidosis, it is important to

differentiate sarcoidal alopecia from DLE so that proper treatment can be initiated, thereby avoiding the

potential long-term sequelae. We herein present a case of Taiwanese woman with scalp sarcoidal alopecia

mimicking DLE.

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Introduction

Sarcoidosis is an idiopathic systemic granulomatous disease in which non-caseating granulomatous

inflammation can occur in any organ. The skin is affected in around 25% of cases, and the majority of

them are African-American.1 Involvement of the scalp is rare and may lead to cicatricial alopecia as a

result of the destruction of the hair follicles by the granulomatous formation.1 Clinically, sarcoidosis may

present as papules, nodules, or plaques, and in some cases resembling discoid lupus erythematosus (DLE)

or necrobiosis lipoidica.2-6

Case Report

This 57-year-old female patient was quite well in the past. This time, she suffered from alopecia on the

fronto-parietal scalp for 4-5 years (Figure 1). There are several bean to coin-sized depressed ulcers with

surrounding violaceous to erythematous hue and telangiectasia on the right fronto-parietal scalp. An

incisional biopsy was performed under the suspicion of DLE. The pathology showed non-caseating

granulomatus inflammation involving superficial and deep dermis (Figure 2). Therefore, the diagnosis of

sarcoidal alopecia was confirmed. The immunologic profiles including antinuclear antibody,

anti-extracted nuclear antigen (ENA) antibody, C3 and C4 were within normal range but chest plain film

and computed tomography revealed pulmonary sarcoidosis with lymphadenopathies (Figure 3). The lung

function tests showed normal spirometry and diffusion capacity. Other blood tests such as complete blood

cell counts, serum aspartate aminotransferase, alanine aminotransferase, creatinine, blood urea nitrogen,

sodium, potassium and calcium levels were all within normal limits. Because the patient refused

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treatment with systemic corticosteroids, she was treated with oral hydroxychloroquine 400 mg daily and

intralesional injections of triamcinolone 10 mg monthly. A total of 9 intralesional triamcinolone

injections were performed from October 2009 to December 2011 in a monthly base except the 6 months

from April to September of 2011. Because of significant improvement and hair regrowth, we

discontinued the local corticosteroid treatment since January 2012. The dosage of oral

hydroxychloroquine was also tapered to 200 mg daily since February 2012 and the disease showed no

deterioration after 3 months (Figure 4). Serial computed tomographic examinations of the chest from

2009 to 2012 showed stable of the disease and the lung function tests remaining normal.

Discussion

Sarcoidal alopecia is rare and predominantly affects African American women.1 It is a form of

secondary scarring alopecia and can have several morphologies. Most commonly, sarcoidal plaques

located on the fronto-parietal facial region may extend into the scalp, thus leading to hair loss. Such

plaques are the type of sarcoidal alopecia that most closely resembles DLE.1-4

On the scalp, sarcoidosis can start as an atrophic, red, scaling or ulcerative area of alopecia. 1 The

typical lesion of classic DLE is a well-circumscribed, erythematous, slightly scaly, atrophic plaque and

may be ulcerative occasionally.2 Follicular plugging can be appreciated under dermoscopy, in both

sarcoidal alopecia and DLE.5 Differential diagnosis of sarcoidal alopecia versus DLE could be made by

histopathologic examination. Sarcoidal alopecia shows classic sarcoidal granulomas in the dermis.1-4 In

contrast, DLE is characterized by follicular plugging, epidermal basal cell vaculopathy, as well as

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superficial and deep perivascular and periadnexal lymphocytic infiltrate. 2

In patients with sarcoidal alopecia, the patient almost has other cutaneous lesions, and the vast

majority of cases will demonstrate systemic involvement. 1 About 30% of patients with the initial form of

cutaneous sarcoidosis will develop its systemic form within months to several years of diagnosis. 6

Therefore, it is recommended that any patient with cutaneous sarcoidosis be screened for systemic lesions,

even if there are no clinical complaints of systemic involvement at initial visits. Several diagnostic studies

can be performed in the workup of sarcoidosis, including chest x-ray, chest computed tomography (CT)

and pulmonary function tests.

1, 7-17

Review of the English revealed a total of 47 reported cases of scalp sarcoidosis, including ours.

A female predominance was noted among these patients (35/40). Where race was mentioned, 23 of 36

patients were African-American and 3 of them were Chinese. Many of them (32 of 37) exhibited

extracutaneous involvement and lung was the most frequent involved site. This review documents diverse

morphologies of scalp sarcoidosis. Most of these cases showed scarring alopecia, but cases of

non-scarring alopecia have also been reported.1,10 Sarcoidal alopecia may resemble DLE, lichen

planopilaris, pseudopelade of Brocq, necrobiosis lipoidica, morphea or alopecia neoplastica, of which

DLE is the most confusing one. A comparison of sarcoidal alopecia, DLE, lichen planopilaris, and

pseudopelade of Brocq was given as Table 1.

The standard treatment for sarcoidosis is systemic corticosteroids. Topical corticosteroids are often

ineffective in treating sarcoidal alopecia because of their inadequate depth of penetration into the skin.

Combination of antimalarial therapy (hydroxychloroquine plus quinacrine or chloroquine plus quinacrine)

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can be of value after single-agent antimalarial therapy has failed.1 The rationale of using antimalarial

therapy in cutaneous sarcoidosis is based on the ability of these agents to inhibit antigen processing and

presentation by antigen presenting cells to CD4+ T cells.20 One of the initial steps in granuloma formation

is antigen processing and presentation, and antimalarials may function to raise the pH within lysosomes,

thus preventing assembly of MHC-peptide complexes and transport to the cell surface. Without antigen

processing and presentation via MHC-peptide complexes, T cells are not activated to propagate

granuloma formation. 20 These agents have a relatively long history of use in the treatment of sarcoidosis

and are considered standard therapy, typically in conjunction with corticosteroids or for patients in whom

corticosteroids are neither desirable nor necessary for long-term treatment. Based on reported clinical

experience, the primary benefit of antimalarials appears to be their ability to suppress cutaneous lesions.21

Due to the disfigurement of the scalp lesions and the patient��s hesitance to the systemic corticosteroids

treatment, oral hydroxychloroquine and intralesional corticosteroids were administered simultaneously.

DLE and several other diseases that resemble cutaneous sarcoidosis are relatively benign diseases.

Because of its characteristic clinical appearance and low association with systemic lupus erythematosus,

therapy for DLE is often administered without histologic confirmation.2 This precludes identification of

its close clinical stimulant, cutaneous sarcoidosis. We stress the importance of a skin biopsy to confirm

the diagnosis of DLE on the scalp and to exclude sarcoidal alopecia. Because of the systemic and

progressive nature of sarcoidosis, it is critical that this distinction is made and the patient treated

accordingly. 2 Even though our patient did not receive any systemic corticosteroid treatment due to

personal reasons, the identification of sarcoidosis did help disease monitoring and appropriate treatment if

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disease progression in the future.

Conclusion

Cicatricial alopecia presents a diagnostic challenge to clinicians. In particular, lesions of cutaneous

sarcoidosis of the scalp may resemble DLE. Pathological examination is required to make the correct

diagnosis, which leads to effective treatment.