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CASE REPORT
Sarcoidal alopecia mimicking discoid lupus erythematosus: Report of
a case and review of the literature
Chia-Fen Tsai, Hsing-Chuan Lee, Chia-Yu Chu
*
Department of Dermatology, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, Taiwan
article info
Article history:
Received: Aug 8, 2012
Revised: Feb 11, 2013
Accepted: Feb 21, 2013
Keywords:
sarcoidal alopecia
discoid lupus erythematosus
abstract
Sarcoidal alopecia is a subtype of plaque-forming cutaneous sarcoidosis that may resemble discoid lupus
erythematosus (DLE). Because the clinical appearance of the two lesions is similar, the correct diagnosis
may be missed. The systemic involvement and progressive nature of sarcoidosis, make it important to
differentiate sarcoidal alopecia from DLE, so that proper treatment can be initiated and potential long-
term sequelae avoided. We present the case of a 57-year-old Taiwanese woman with sarcoidal alope-
cia of the scalp that mimicked DLE.
Copyright Ó2013, Taiwanese Dermatological Association.
Published by Elsevier Taiwan LLC. All rights reserved.
Introduction
Sarcoidosis is an idiopathic systemic granulomatous disease, in
which noncaseating granulomatous inflammation can occur in any
organ. The skin is affected in about 25% of cases, and the majority of
cases occur in black women.
1
The scalp is rarely involved, and the
inflammation may lead to cicatricial alopecia as a result of the
destruction of hair follicles by the granulomatous formation.
1
Clinically, sarcoidosis may present as papules, nodules, or pla-
ques, and in some cases may resemble discoid lupus erythematosus
(DLE) or necrobiosis lipoidica.
2e6
Case report
A 57-year-old Taiwanese female had been very healthy most of her
life. However, over the past 4e5 years, she had developed painful
ulcerative wounds with alopecia on her frontoparietal scalp
(Figure 1). She had not been treated for the wounds or alopecia
prior to visiting our hospital. On examination, we saw several bean-
to coin-sized depressed ulcers surrounded by a violaceous to
erythematous hue, and telangiectasia on the right frontoparietal
scalp. No other skin lesions were noted.
DLE was suspected, and an incisional biopsy was performed. The
pathology report showed noncaseating granulomatous inflammation
involving the superficial and deep dermis (Figure 2). The granulomas
were composed of epithelioid cells and occasional giant cells, rich in
asteroid bodies. Results of direct immunofluorescence tests for
immunoglobulin A (IgA), IgG, IgM, complement component 3 (C3),
fibrinogen, and complement C1q were all negative. Periodic acid
Schiff, Fite, and acid-fast stains did not reveal any pathogens, and the
Venereal Disease Research Laboratory (VDRL) test was negative.
Therefore, the diagnosis of sarcoidal alopecia was confirmed.
The immunologic profiles, including antinuclear antibody, anti-
extracted nuclear antigen (ENA) antibody, C3, and C4 were all
within normal ranges. However, plain chest films and computed
tomography (CT) scans revealed pulmonary sarcoidosis with
lymphadenopathy (Figure 3). Lung function tests showed normal
spirometric values and diffusing capacity. Ophthalmologic exami-
nation ruled out any ocular involvement. The results of other blood
tests, such as complete blood cell counts, and measurement of
serum aspartate aminotransferase, alanine aminotransferase,
creatinine, blood urea nitrogen, sodium, potassium and calcium
levels, were all within normal limits.
Because the patient refused treatment with systemic cortico-
steroids, she was treated with oral hydroxychloroquine, 400 mg
daily, and intralesional injections of triamcinolone, 10 mg once a
month. She received a total of nine intralesional triamcinolone in-
jections from October 2009 to December 2011; these injections
were given on a monthly basis, except during the 18 months from
April 2010 to September 2011.
Because the patient had significant overall improvement and
regrowth of hair, we discontinued local corticosteroid treatment in
January 2012. The dosage of oral hydroxychloroquine was also
tapered to 200 mg daily (beginning February 2012) and the disease
Conflicts of interest: The authors declare thatthey have no financial or non-financial
conflicts of interest related tothe subject matter or materials discussed in this article.
*Corresponding author. Department of Dermatology, National Taiwan University
Hospital, No. 7, Chung Shan South Road, Taipei 100, Taiwan. Tel.: þ886 2 2356 2141;
fax: þ886 2 2393 4177.
E-mail address: chiayu@ntu.edu.tw (C.-Y. Chu).
Contents lists available at SciVerse ScienceDirect
Dermatologica Sinica
journal homepage: http://www.derm-sinica.com
1027-8117/$ esee front matter Copyright Ó2013, Taiwanese Dermatological Association. Published by Elsevier Taiwan LLC. All rights reserved.
http://dx.doi.org/10.1016/j.dsi.2013.02.004
DERMATOLOGICA SINICA 32 (2014) 43e46
showed no deterioration after 3 months (Figure 4). Serial CT ex-
aminations of the chest from 2009 to 2012 showed that the disease
had stabilized, and lung function tests remained normal.
Discussion
Sarcoidal alopecia is a rare manifestation of cutaneous sarcoidosis
that predominantly affects black women.
1
It is a form of secondary
cicatricial alopecia and can have variable morphologies. Most
commonly, cutaneous sarcoidosis on the frontoparietal facial re-
gion may extend into the scalp, which may lead to hair loss. Such
lesions are the type of sarcoidal alopecia that most mimics DLE
clinically.
1e4
On the scalp, sarcoidosis can manifest as atrophic, erythema-
tous, scaly, or ulcerative areas of alopecia.
1
The typical appearance
of DLE is of well-circumscribed, erythematous, scaly, atrophic pla-
ques, that may occasionally also be ulcerative.
2
In both situations,
follicular plugging can be observed on dermoscopy.
5
The differen-
tial diagnosis of sarcoidal alopecia and DLE can be made by histo-
pathologic examination. Sarcoidal alopecia shows classic naked
granulomas in the dermis.
1e4
In contrast, DLE is characterized by
follicular plugging, epidermal atrophy, vacuolar degeneration of
basal keratinocytes, and basement membrane thickening, as well as
superficial and deep perivascular and periadnexal lymphocytic
infiltrates.
2
Patients with sarcoidal alopecia almost always have other
cutaneous lesions, and the vast majority of cases will also demon-
strate 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 sys-
temic lesions, even if there are no clinical complaints of systemic
involvement at initial visits. Several diagnostic studies can be per-
formed during the workup of sarcoidosis, including chest films,
chest CT scans, and pulmonary function tests.
A review of the English literature revealed 47 reported cases of
scalp sarcoidosis, including ours.
1,3,7e16
In these studies, patients
were predominantly female (35/40). Where race was mentioned,
Figure 1 Cicatricial alopecia with several bean- to coin-sized depressed ulcers. A
surrounding violaceous to erythematous hue can be seen on the patient’s right fron-
toparietal scalp.
Figure 2 Pathological examination revealed noncaseating granulomatous inflammation involving superficial and deep dermis. Many asteroid bodies were found. (A) Original
magnification 40, hematoxylin and eosin stain; (B) original magnification 100, hematoxylin and eosin stain.
Figure 3 Plain chest film shows bilateral hilar enlargement.
Figure 4 After treatment, the alopecia was nearly resolved.
C.-F. Tsai et al. / Dermatologica Sinica 32 (2014) 43e4644
23 of 36 patients were black and three patients were Chinese. Many
of the patients (32 of 37) exhibited extracutaneous involvement,
and the lung was the most frequently involved site. Other extrac-
utaneous manifestations included lymphadenopathies (18 of 37
patients), musculoskeletal involvement (4 of 37 patients), hep-
atosplenomegaly (3 of 37 patients), and ocular involvement (2 of 37
patients).
Our review of cases documented diverse morphologies of scalp
sarcoidosis, including papules, nodules, indurative plaques, scales,
and ulcers. Most of these cases showed scarring alopecia, but some
cases of non-scarring alopecia have also been reported.
1,3
Sarcoidal
alopecia may resemble DLE, lichen planopilaris (follicular lichen
planus), pseudopelade of Brocq (alopecia cicatrisata), necrobiosis
lipoidica, morphea or alopecia neoplastica. DLE is the most
confusing form. A comparison of the features of sarcoidal alopecia
and DLE is presented in Table 1.
Given the limited clinical data available, it is difficult to draw
definite conclusions about responses to therapy, prognosis, and
outcome for patients with sarcoidal alopecia. Some authors have
reported having poor responses with multiple treatments,
including intralesional and/or systemic corticosteroids, antimalar-
ials, and azathioprine. The overall response to treatment appears
poor, but more information and analysis of more cases are needed
before accurate conclusions can be made.
The standard treatment for sarcoidosis is systemic corticoste-
roids. Topical corticosteroids are often ineffective in treating sar-
coidal alopecia, because of their inadequate depth of penetration
into the skin. Combination antimalarial therapy (hydroxy-
chloroquine plus quinacrine or chloroquine plus quinacrine) can be
valuable 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 CD4þT cells.
17
Antima-
larials may raise the pH within lysosomes, thus preventing assembly
of major histocompatibility complex (MHC)epeptide complexes
and transport to the cell’s surface. Without antigen processing and
presentation via MHCepeptide complexes, noT cells are activated to
promote granuloma formation.
17
Antimalarial agents have a rela-
tively long history of use in the treatment of sarcoidosis, and are
regarded as standard therapy. Typically, they are used in combina-
tion with corticosteroids, or singly for patients in whom cortico-
steroids are undesirable or unnecessary for long-term treatment.
Based on reported clinical experience, the primary benefit of anti-
malarials appears to be their ability to suppress the formation of
cutaneous lesions.
18
In our case, due to the disfigurement from the
scalp lesions and our patient’s hesitance to be treated with systemic
corticosteroids, we administered hydroxychloroquine and intrale-
sional corticosteroids simultaneously.
DLE and several other diseases that resemble cutaneous
sarcoidosis are relatively benign. Because of its characteristic clin-
ical appearance and infrequent association with systemic lupus
erythematosus, therapy for DLE is often administered without
histologic confirmation of lesions.
2
This precludes identification of
its close clinical simulation, cutaneous sarcoidosis. In our practice,
we stress the importance of performing 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 that the patient be treated
accordingly.
2
Even though our patient did not receive any systemic
corticosteroid treatment because of her personal preference, the
identification of sarcoidosis did help; disease monitoring would
enable us to provide appropriate treatment, should the disease
progress.
Cicatricial alopecia presents a diagnostic challenge for clinicians,
particularly because the lesions of cutaneous sarcoidosis of the
scalp may resemble DLE. Pathological examination is required to
make the correct diagnosis, which then leads to effective treatment.
References
1. Katta R, Nelson B, Chen D, Roenigk H. Sarcoidosis of the scalp: a case series and
review of the literature. J Am Acad Dermatol 2000;42:690e2.
2. Henderson CL, Lafleur L, Sontheimer RD. Sarcoidal alopecia as a mimic of
discoid lupus erythematosus. J Am Acad Dermatol 2008;59:143e5.
3. Cho HR, Shah A, Hadi S. Systemic sarcoidosis presenting with alopecia of the
scalp. Int J Dermatol 2004;43:520e2.
4. Ball NJ, Kho GT, Martinka M. The histologic spectrum of cutaneous sarcoidosis:
a study of twenty-eight cases. J Cutan Pathol 2004;31:160e8.
5. Torres F, Tosti A, Misciali C, Lorenzi S. Trichoscopy as a clue to the diagnosis of
scalp sarcoidosis. Int J Dermatol 2011;50:358e61.
6. Tchernev G. Cutaneous sarcoidosis: ‘‘the great imitator’’: etiopathogenesis,
morphology, differential diagnosis, and clinical management. Am J Clin Der-
matol 2006;7:375e82.
7. Chao SC, Yan JJ, Lee JY. Cutaneous sarcoidosis among Taiwanese. J Formos Med
Assoc 2000;99:317e23.
8. Harman KE, Calonje E, Robson A, Black MM. Sarcoidosis presenting as a scarring
alopecia resembling necrosis lipoidica. Clin Exp Dermatol 2003;28:565e6.
9. Akhdari N, Skalli HD, Lakhdar H. Erythematous lesions on the scalp. Sarcoidosis.
Arch Dermatol 2004;140:1003e8.
10. Chong WS, Tan HH, Tan SH. Cutaneous sarcoidosis in Asians: a report of 25
patients from Singapore. Clin Exp Dermatol 2005;30:120e4.
11. Mahajan VK, Sharma NL, Sharma RC, Sharma VC. Cutaneous sarcoidosis: clinical
profile of 23 Indian patients. Indian J Dermatol Venereol Leprol 2007;73:16e21.
12. Cay F, Sezer I, Kacar C, Butun B, Tuncer T. Musculoskeletal sarcoidosis with a
dermatologic clue for diagnosis: a case report. Rheumatol Int 2007;27:497e9.
13. Rajasekharan P, Narurkar S, Patel K, Rajpal A. Asteroid bodies in a lymph node
aspirate. A case report. Acta Cytol 2007;51:66e7.
14. La Placa M, Vincenzi C, Misciali C, Tosti A. Scalp sarcoidosis with systemic
involvement. J Am Acad Dermatol 2008;59:S126e7.
Table 1 A comparison of sarcoidal alopecia and discoid lupus erythematosus (DLE).
Sarcoidal alopecia DLE
Epidemiology Women >men
Blacks
Women >men
Blacks
Clinical features Atrophic, red, scaling or ulcerative areas of alopecia
A rare manifestation of cutaneous sarcoidosis
Frequent extracutaneous involvement; the
lung is the most frequently involved site
Relatively poor response to treatments
Erythematous scaly plaques with follicular plugs, telangiectasia, atrophy
and pigment changes; activity in the center of the alopecic patch
Frequently noted on the face, scalp, ears, V-neck area, and extensor sides of arms
5% of patients presenting with classic DLE lesions subsequently develop evidence of SLE
With treatment, skin disease can be largely controlled
Histopathology Noncaseating granulomas in the
dermis, with destruction of hair follicles and fibrosis
Lymphocytic infiltrate centered on infundibulum and isthmus
Interfollicular epidermal inflammation and thickened basement membrane
Interface dermatitis (vacuolar >lichenoid)
Superficial and deep perivascular and perieccrine inflammation
Dermal mucin
DIF: granular linear IgG and C3 along the basement membrane
Therapy Topical intralesional corticosteroids
Prednisone, hydroxychloroquine, methotrexate
Infliximab
Topical intralesional corticosteroids
Hydroxychloroquine, prednisone, topical tacrolimus, tazarotene, imiquimod
Isotretinoin
DIF ¼direct immunofluorescence; SLE ¼systemic lupus erythematosus.
C.-F. Tsai et al. / Dermatologica Sinica 32 (2014) 43e46 45
15. Collin B, Rajaratnam R, Lim R, Lewis H. A retrospective analysis of 34 patients
with cutaneous sarcoidosis assessed in a dermatology department. Clin Exp
Dermatol 2010;35:131e4.
16. Lee JY, Chao SC, Yang MH, Yan JJ. Sarcoidosis in Taiwan: clinical characteristics
and atypical mycobacteria. J Formos Med Assoc 2002;101:749e55.
17. Doherty CB, Rosen T. Evidence-based therapy for cutaneous sarcoidosis. Drugs
2008;68:1361e83.
18. Siltzbach LE, Teirstein AS. Chloroquine therapy in 43 patients with intra-
thoracic and cutaneous sarcoidosis. Acta Med Scand 1964;176(Suppl 425):
302e6.
C.-F. Tsai et al. / Dermatologica Sinica 32 (2014) 43e4646
