pmc logo image
Logo of bumcprocBaylor University Medical Center ProceedingsAbout the JournalBaylor Health Care SystemSubmit a Manuscript

Formats:

Proc (Bayl Univ Med Cent). 2001 January; 14(1): 101–103.
PMCID: PMC1291318
Hair loss and plaquelike skin lesions
Jennifer Clay Cather, MDcorresponding author1 and M. Alan Menter, MD1
1From the Division of Dermatology, Department of Internal Medicine, Baylor University Medical Center, Dallas, Texas.
corresponding authorCorresponding author.
Corresponding author: Jennifer Clay Cather, MD, 3600 Gaston Avenue, Suite 651, Dallas, Texas 75246.
A healthy 35-year-old African American woman presented for evaluation of hair loss (Figure (Figure11Figure 1). Examination of her scalp revealed areas of alopecia with absent hair follicles, erythematous papules, and a few pustules, consistent with a scarring (cicatricial) form of alopecia. Additionally, there were hypopigmented lesions on her arms (Figure (Figure22Figure 2) and targetoid plaquelike lesions on her legs (Figure (Figure33Figure 3).
Figure 1
Figure 1
Figure 1
Posterior scalp with areas of scarring alopecia and erythematous papules and plaques
Figure 2
Figure 2
Figure 2
Hypopigmented macules on the arm.
Figure 3
Figure 3
Figure 3
Lower legs with targetoid plaques.
What is your diagnosis?
DIAGNOSIS: Sarcoidosis.
Sarcoidosis is a multisystem granulomatous disease of unknown etiology that involves the lungs in >90% of patients, eyes in 25% to 50%, lymph nodes in 75%, and skin in 25% to 30% (14). Pure cutaneous involvement is seen in approximately 25% of patients (5). The mucocutaneous lesions of sarcoidosis are diverse (Table) and can be divided into those that are specific and those that are nonspecific. Specific lesions demonstrate histological evidence of noncaseating granulomas in the dermis and are associated with chronic disease. A recent review of cutaneous sarcoidosis found that 73% of the patients had specific lesions at the beginning of their disease, with 70% of those patients having systemic manifestations concomitantly. The remaining 30% developed systemic disease between 6 months and 3 years later (17, 28). Clinically, specific lesions include a wide variety of papules, plaques, lupus pernio (red-purple papules and plaques on the face and fingers), pits/spicules, and alopecia. Other specific lesions may be verrucous, angiolupoid (having a prominent vascular or telangiectatic component), ulcerative, mutilating, pustular, or erythrodermic. Nonspecific lesions do not reveal granulomatous inflammation in the dermis but are associated with systemic involvement of sarcoidosis. Examples of nonspecific lesions include erythema nodosum (17%), clubbing, calcinosis cutis, and erythema multiforme. Usually, nonspecific lesions such as erythema nodosum are associated with acute disease and portend a more favorable prognosis.
Table
Table
Mucocutaneous manifestations of sarcoidosis
Several medically important entities are associated with sarcoidosis. Lofgren's syndrome is characterized by bilateral hilar lymphadenopathy, fever, arthralgias, erythema nodosum, and uveitis. Typically, 80% of these cases resolve within 2 years. Uveoparotid fever (Heerfordt's syndrome) consists of parotid and lacrimal gland involvement, facial nerve palsy, uveitis, and fever; in addition, central nervous system involvement is frequently present. Cutaneous lesions on the nose are commonly associated with sarcoidosis of the upper respiratory tract.
Histology
On histologic examination, specific lesions have well-defined epithelioid granulomas with minimal, if any, necrosis or inflammation (i.e., naked granulomas). Giant cells may be present. Three types of inclusion bodies have been seen but may not be specific for sarcoidosis: 1) Schaumann's bodies (calcium carbonate, phosphate, and iron), 2) asteroid bodies (lipoprotein), and 3) residual bodies (lipomucoprotein granules) (29).
Laboratory evaluation
Increased calcium has been found in blood and urine of patients with sarcoidosis. Additionally, increased angiotensin-converting enzyme levels have been reported; however, this can also be seen in patients with diabetes, alcoholic liver disease, various infections like leprosy, and Gaucher's disease. The Kveim- Siltzbach test, an intradermal injection of heat-sterilized sarcoid tissue followed by a biopsy at the injection site 6 weeks later, shows granulomas in 80% of patients with active disease (2).
Differential diagnosis
Sarcoidosis, like syphilis, has a great variety of cutaneous manifestations. The most common mimics include the following:
  • Infections (leprosy, lupus vulgaris [cutaneous tuberculosis], leishmaniasis, syphilis)
  • Inflammatory skin diseases (lichen planus, granuloma annulare, necrobiosis lipoidica diabeticorum, lupus erythematosus, rosacea)
  • Neoplasms (cutaneous lymphomas and leukemias)
Treatment
There is no cure for sarcoidosis. Treatment is dictated by the extent of involvement. Cutaneous sarcoidosis has been treated successfully with systemic, intralesional, and topical steroids. Other agents employed include antimalarials (30), retinoids, and immunosuppressives such as methotrexate (31). For more disfiguring cutaneous lesions, even in the absence of systemic disease, systemic therapy is frequently indicated.
1. DeRemee RA. Sarcoidosis. Mayo Clin Proc. 1995;70:177–181. [PubMed]
2. Kerdel FA, Moschella SL. Sarcoidosis. An updated review. J Am Acad Dermatol. 1984;11:1–19. [PubMed]
3. Zax RH, Callen JP. Sarcoidosis. Dermatol Clin. 1989;7:505–515. [PubMed]
4. Samtsov AV. Cutaneous sarcoidosis. Int J Dermatol. 1992;31:385–391. [PubMed]
5. Hanno R, Needelman A, Eiferman RA, Callen JP. Cutaneous sarcoidal granulomas and the development of systemic sarcoidosis. Arch Dermatol. 1981;117:203–207. [PubMed]
6. Greer KE, Harman LE, Jr, Kayne AL. Unusual cutaneous manifestations of sarcoidosis. South Med J. 1977;70:666–668. [PubMed]
7. Golitz LE, Shapiro L, Hurwitz E, Stritzler R. Cicatricial alopecia of sarcoidosis. Arch Dermatol. 1973;107:758–760. [PubMed]
8. Rasmussen JE. Sarcoidosis in young children. J Am Acad Dermatol. 1981;5:556–570.
9. Okano M, Nishimura H, Morimoto Y, Maeda H. Faint erythema. Another manifestation of cutaneous sarcoidosis? Int J Dermatol. 1997;36:681–684. [PubMed]
10. Beacham BE, Schuldenfrei J, Julka SS. Sarcoidosis presenting with erythema multiforme–like cutaneous lesions. Cutis. 1984;33:461–463. [PubMed]
11. Hannuksela M. Erythema nodosum. Clin Dermatol. 1986;4:88–95. [PubMed]
12. Wigley JEM, Musso LA. A case of sarcoidosis with erythrodermic lesions: treatment with calciferol. Br J Dermatol. 1951;63:398. [PubMed]
13. Clayton R, Breathnach A, Martin B, Feiwel M. Hypopigmented sarcoidosis in the Negro. Report of eight cases with ultrastructural observations. Br J Dermatol. 1977;96:119–125. [PubMed]
14. Cather JC, Cohen PR. Ichthyosiform sarcoidosis. J Am Acad Dermatol. 1999;40:862–865. [PubMed]
15. Morrison JGL. Sarcoidosis in a child, presenting as an erythroderma with keratotic spines and palmar pits. Br J Dermatol. 1976;95:93–97. [PubMed]
16. Gange RW, Smith NP, Fox ED. Eruptive cutaneous sarcoidosis of unusual type. Report of two cases without radiologically demonstrable lung involvement. Clin Exp Dermatol. 1978;3:299–306. [PubMed]
17. Mana J, Marcoval J, Graells J, Salazar A, Peyri J, Pujol R. Cutaneous involvement in sarcoidosis. Relationship to systemic disease. Arch Dermatol. 1997;133:882–888. [PubMed]
18. Fong YW, Sharma OP. Pruritic maculopapular skin lesions in sarcoidosis. An unusual clinical presentation. Arch Dermatol. 1975;111:362–364. [PubMed]
19. Morrison JG. Sarcoidosis in the Bantu. Necrotizing and mutilating forms of the disease. Br J Dermatol. 1974;90:649–655. [PubMed]
20. Mann RJ, Allen BR. Nail dystrophy due to sarcoidosis. Br J Dermatol. 1981;105:599–601. [PubMed]
21. Kalb RE, Grossman ME. Pterygium formation due to sarcoidosis. Arch Dermatol. 1985;121:276–277. [PubMed]
22. Elgart ML. Cutaneous sarcoidosis: definitions and types of lesions. Clin Dermatol. 1986;4:35–45. [PubMed]
23. Kindler T. Small nodular disseminated sarcoid, localized to light-exposed areas. Br J Dermatol. 1955;67:149–152. [PubMed]
24. Veien NK, Stahl D, Brodthagen H. Cutaneous sarcoidosis in Caucasians. J Am Acad Dermatol. 1987;16:534–540. [PubMed]
25. Gross MD, Andriacchi F, Gordon R, Maddox D. Nodular subcutaneous sarcoidosis. Arch Dermatol. 1977;113:1442–1443. [PubMed]
26. Verdegem TD, Sharma OP. Cutaneous ulcers in sarcoidosis. Arch Dermatol. 1987;123:1531–1534. [PubMed]
27. Shmunes E, Lantis LR, Hurley HJ. Verrucose sarcoidosis. Arch Dermatol. 1970;102:665–669. [PubMed]
28. Olive KE, Kataria YP. Cutaneous manifestations of sarcoidosis. Relationships to other organ system involvement, abnormal laboratory measurements, and disease course. Arch Intern Med. 1985;145:1811–1814. [PubMed]
29. Rabinowitz LO, Zaim MT. A clinicopathologic approach to granulomatous dermatoses. J Am Acad Dermatol. 1996;35:588–600. [PubMed]
30. Jones E, Callen JP. Hydroxychloroquine is effective therapy for control of cutaneous sarcoidal granulomas. J Am Acad Dermatol. 1990;23:487–489. [PubMed]
31. Veien NK, Brodthagen H. Cutaneous sarcoidosis treated with methotrexate. Br J Dermatol. 1977;97:213–216. [PubMed]

See more articles cited in this paragraph
See more articles cited in this paragraph
See more articles cited in this paragraph
See more articles cited in this paragraph