4.
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3.4.
Diagnosis: Sarcoidosis with scalp and joint involvement
Histopathology:
The biopsy (Figs. 3 and 4) revealed several non-caseating granulomas in the papillary dermis. Special stains for acid-fast bacteria and fungus were negative. The dermatopathologist concluded that the histology was compatible with sarcoidosis.
The patient was treated with hydroxychoroquine 200 milligrams twice daily and clobetasol propionate 0.05% gel applied to the scalp lesion twice daily. Upon return to the dermatology clinic at four-mouth follow up, there was significant re-growth of hair and a diminution in the stiffness and pain of his hands.
Discussion:
Sarcoidosis is an idiopathic multisystem granulomatous disease. It is most common in African Americans and Northern European Caucasians. The disease prevalence is 40 per 100,000 among African Americans and 5 per 100,000 among white Americans2. Geographic variation has also been noted, with reported incidence in Spain of 0.04 per 100,000 and Sweden of 64 per 100,0001. The disease has protean manifestations. The lymph nodes are nearly always affected. The lungs, spleen, liver, bone marrow, heart, musculoskeletal system and skin are also commonly affected. Sarcoidosis likely has an immune mediated etiology (1, 3).
Approximately 25% of patients with sarcoidosis exhibit cutaneous manifestations. The most common cutaneous manifestation in black patients is a waxy, translucent papular eruption over the face, lids, nasolabial folds, upper back and around the orbits(4). Sarcoidosis has a broad range of less commonly reported cutaneous presentations, including follicular papules, ichthyotic scaling, ulcers, scaly plaques, verrucous papules, and hypopigmentation7.
Alopecia is a rare manifestation of sarcoidosis; few cases have been reported in the past three decades (8, 9,10,11). The scalp lesions may resemble necrobiosis lipoidica, discoid lupus erythematosus, lichen planopilaris, or pseudopelade of Brocq.
Arthritis occurs in only 10-15% of patients with sarcoidosis. The most common form of arthritis is an acute form that presents early in the course of the disease. Chronic arthritis, as in our patient, is less common and typically manifests itself as dactylitis. Our patient's musculoskeletal sarcoidosis provided a clue to the nature of his cutaneous disease as bony changes are most often seen in black males with chronic cutaneous sarcoidosis (1,2).
Frequently, the thorax will be involved yet the patient may be asymptomatic as was he case in tis patient. Hypercalcemia or hypercaliuria is present in the vast majority of patients. The Angiotensin-Converting enzyme level is elevated in 75% of patients, but it is not universally useful in diagnosis (1,2,4).
Many therapies for cutaneous sarcoidosis have been employed. However, the mainstays of therapy are steriods and antimalarials. Corticosteriods can be administered systemically, intralesionally, or topically. Antimalarials are most useful for the treatment of chronic skin lesions. Chloroquine (250 mg) or Hydroxychloroquine (200 mg) can be administered twice daily (4).
References:
1. Fanburg BL. Sarcoidosis. In: Cecil Textbook of Medicine (Bennett JC, Plum F, eds.), 20th edn. Philadelphia: W.B. Saunders; 1996:431-436.
2. Hellman DB. Sarcoidosis. In: Primer on the Rheumatic Diseases (Klippel JH, ed.), 11th edn. Atlanta: Arthritis Foundation; 1997:325-327.
3. von Lichtenberg F. Sarcoidosis (Or Boeck's Sarcoid). In: Robbins Pathologic Basis of Disease (Cotran RS, Kumar V, Robbins SL, eds.), 4th edn. Philadelphia: WB Saunders; 1996:427-429.
4. Sharma OP. Sarcoidosis of the Skin. In: Fitzpatrick's Dermatology in General Medicine (Freedberg IM, Eisen AZ, Wolff K, etal., eds.), 5th ed. New York: McGraw-Hill; 1999:2099 2106.
5. Kirsner RS, Kerdel FA. Granulomatous Inflammation. In Cutaneous Medicine and Surgery (Arndt KA, LeBoit PE, Robinson JK, etal., eds.), 1st edn. Vol. 1. Philadelphia: WB Saunders, 1996; 433-437.
6. Gawkrodger DJ. Sarcoidosis. In: Textbook of Dermatology (Champion RH, Burton JL, Burns DA etal. eds.), 6TH edn. Vol. 3. Oxford: Blackwell Science, 1998; 2685.
7. Zax RH, Callen JP. Granulomatous Reactions. In: Sams WM Jr., Lynch PJ, eds. Principles and Practices of Dermatology. 2nd ed. New York, NY: Churchill Livingstone; 1996:629-632.
8. Takahashi H, Mori M, Muraoka S, et al. Sarcoidosis Presenting as a Scarring Alopecia: Report of a Rare Cutaneous Manifestation of Systemic Sarcoidosis. Dermatol. 1996;193:144-146.
9. Golitz LE, Shapiro L, Hurwitz e, et al. Cicatrial Alopecia of Sarcodosis. Arch Dermatol. 1973;107:758-760.
10. Maurice PD, Goolamali SK. Sarcoidosis of the scalp presenting as scarring alopecia. Br J Dermatol. 1988;110(suppl 33):116-117
11. Bluefarb SM, Szymanski FJ, Rostenberg A Jr. Sarcoidosis as a Cause of Patchy Alopecia. Arch Dermatol. 1955;71:602-604.
12. Strutton G. The Granulomatous Reaction Pattern. In: Skin Pathology (Weedon D. ed.), 1st edn. London: Churchill Livingston, 1998; 161-163.
Maurice PD, Goolamali SK. Sarcoidosis of the scalp presenting as scarring alopecia. Br J Dermatol. 1988;110(suppl 33):116-117
11. Bluefarb SM, Szymanski FJ, Rostenberg A Jr. Sarcoidosis as a Cause of Patchy Alopecia. Arch Dermatol. 1955;71:602-604.
12. Strutton G. The Granulomatous Reaction Pattern. In: Skin
Pathology (Weedon D. ed.), 1st edn. London: Churchill Livingston, 1998;
161-163.
My thanks to Drs. Brent Moody, and Michael Heffernan for their help in
the preparation of this case.
