Sarcoidosis: Cutaneous Lesions and Characteristic Lesions on Arms

November 1, 2008

The asymptomatic lesions on this 63-year-old woman’s forehead had been present for about 4 years, gradually enlarging and sometimes scarring in the center as they progressed peripherally.

Cutaneous Lesions as the Sole ManifestationCharacteristic Lesions on the ArmsAsymptomatic Hyperpigmented Papules as Initial ManifestationErythema Nodosum as the Presenting SymptomSarcoidosis Mimicking Cutaneous Lesions

Cutaneous Lesions as the Sole Manifestation

The asymptomatic lesions on this 63-year-old woman’s forehead had been present for about 4 years, gradually enlarging and sometimes scarring in the center as they progressed peripherally. The diagnosis of sarcoidosis was confirmed by biopsy, the results of which showed islands of epithelioid cells. The only abnormal laboratory finding was a slightly elevated erythrocyte sedimentation rate. Because sarcoidosis can involve almost any body tissue, the patient was screened with care, but no noncutaneous lesions could be found either in bone or in soft tissue. Intrathoracic involvement may produce cough and shortness of breath but may also be asymptomatic and discovered only on a routine chest roentgenogram or during screening prompted by skin lesions. The granulomatous lesions sometimes involve the heart; ocular involvement can lead to blindness.

(Case and photograph courtesy of Dr Wilfred E. Wooldridge.)

Cutaneous Lesions as the Sole ManifestationCharacteristic Lesions on the ArmsAsymptomatic Hyperpigmented Papules as Initial ManifestationErythema Nodosum as the Presenting SymptomSarcoidosis Mimicking Cutaneous Lesions

Characteristic Lesions on the Arms

The lesions seen on the arms of this patient (A) are typical of sarcoidosis, a multisystem granulomatous disease of unknown origin. In addition to the skin, other sites of involvement include the lungs, eyes, liver, spleen, lymph nodes, muscles, nervous system, and exocrine glands. The disorder is most common among northern Europeans and African Americans; onset usually occurs between ages 20 and 40. The diagnosis of sarcoidosis is generally confirmed when clinical and radiographic findings are supported by histological evidence of numerous noncaseating epithelioid cell granulomas or by a positive Kveim skin test. The Kveim granulomatous reaction appears about a month after intradermal injection of extracts of sarcoid spleen or lymph node and is positive in most patients.

Plaques (B), papules, and subcutaneous nodules are frequently seen in patients who have chronic sarcoidosis, which sometimes begins with the fever and arthralgia of erythema nodosum. The prognosis for clinical recovery is greater in patients with hilar adenopathy alone than in those with pulmonary sarcoidosis and is more favorable for whites than for blacks. Serious disability from damage to eyes and lungs is common, but mortality is less than 3%. Low-dose corticosteroids are frequently used to control disabling symptoms.

(Case and photographs courtesy of Dr Charles A. Woods.)

Cutaneous Lesions as the Sole ManifestationCharacteristic Lesions on the ArmsAsymptomatic Hyperpigmented Papules as Initial ManifestationErythema Nodosum as the Presenting SymptomSarcoidosis Mimicking Cutaneous Lesions

Asymptomatic Hyperpigmented Papules as Initial Manifestation

A 22-year-old man was concerned about facial lesions that had appeared over a 1-year period. They were concentrated around his eyes, nose, and mouth and were asymptomatic. He had no dryness or irritation of the eyes. Examination revealed numerous hyperpigmented papules on the nose (A) and in the perioral region as well as bilateral enlargement of the lacrimal glands (B). No other abnormalities were found.

 

A chest film showed bilateral hilar adenopathy. Microscopic examination of a 4-mm punch biopsy specimen of one of the facial lesions revealed a prominent granulomatous, lymphocytic, and histiocytic infiltrate. These findings are consistent with sarcoidosis.



Examination of a tissue specimen is mandatory for confirmation of sarcoidosis. Most commonly, the specimen is obtained from the lung, using fiberoptic bronchoscopy. Samples of tissue other than those from the lung can be used to confirm the diagnosis, as this case illustrates.

(Case and photographs courtesy of Drs Douglas M. Cahn, Mary Lou Courreg, and Jonathan Stuart Crane.)

Cutaneous Lesions as the Sole ManifestationCharacteristic Lesions on the ArmsAsymptomatic Hyperpigmented Papules as Initial ManifestationErythema Nodosum as the Presenting SymptomSarcoidosis Mimicking Cutaneous Lesions

Erythema Nodosum as the Presenting Symptom

A 26-year-old woman was hospitalized because of low-grade fever, mild cough, and erythema nodosum on the anterior surface of both legs (A). The last symptom had been present for 2 weeks. Results of physical examination and laboratory tests were unremarkable; however, a chest film showed bilateral hilar lymphadenopathy (B).

After 2 weeks, the patient’s serum angiotensin-converting enzyme level had increased. The Kveim test was positive for sarcoidosis; the skin test with purified protein derivative was negative for tuberculosis. (The differential diagnosis of sarcoidosis should always include tuberculosis, which has similar clinical manifestations.)

Slightly more women than men are affected by sarcoidosis. Bilateral hilar lymphadenopathy and pulmonary infiltration are the most frequent manifestations.

(Case, photograph, and film courtesy of Drs N. K. Akritidis, D. H. Makrigiannis, and D. Tsolkas.)

Cutaneous Lesions as the Sole ManifestationCharacteristic Lesions on the ArmsAsymptomatic Hyperpigmented Papules as Initial ManifestationErythema Nodosum as the Presenting SymptomSarcoidosis Mimicking Cutaneous Lesions

Sarcoidosis Mimicking Cutaneous Lesions

Sarcoidosis can often be mistaken for other dermatoses, as in the case of this 32-year-old man who had had pulmonary sarcoidosis for 3 years. The slowly developing cutaneous lesions pictured here had first appeared several weeks earlier. The scaly scalp plaques mimic tinea capitis (A), confluent suprapubic papules look like keloidal acne (B), and hypertrophic papules in a scar resemble prurigo nodules (C).

Cutaneous involvement occurs in about 25% of patients with sarcoidosis. In this case, histopathological examination of punch biopsy specimens from the scalp and suprapubic regions revealed the noncaseating granulomatous inflammation consistent with cutaneous sarcoidosis.

 

 

 

 

 

(Case and photographs courtesy of Drs Frederick Wenzel and Eva Simmons-O’Brien.)