CLINICAL HISTORY:

Signs and symptoms:  A 78 year old African American male presents to the dermatology clinic with a nine year history of a progressively expanding, mildly pruritic rash on his neck and face. He states the eruption began on his neck and spread to his face. In addition, he complains of progressive hair loss and a chronic cough. He had not sought previous medical attention for this condition. Past Medical History - Hypertension.
Past Surgical History - none
Allergies - PCN
Family History - non-contributary
Social History - lives with wife; has two sons; denies tobacco and illicit drug use

Previous Treatment: 

Other information:  Current Medication: Adalat 30mg QD

PHYSICAL EXAM:

Examination reveals mildly erythematous scarring alopecia extending from the front of the scalp to the occiput, sparing the temperoparietal areas. The forehead, bilateral cheeks, jawline, and neck reveal multiple discrete , annular rings of grouped, firm papules with central atrophy. There are no other suspicious lesions noted on the rest of his body.

LABORATORY TESTS:

All labs within normal limits except for an elevated ACE level of 92 IU/L.
CXR reveals bilateral hilar adenopathy

DERMATOHISTOPATHOLOGY:

Microscopic description: Biopsy of scalp: Superficial and deep granulomatous perifolliculitis.
Special stains negative for microorganisms.
Biopsy of Left Neck: Noncaseating granulomatous dermatitis.
Special stains negative for microorganisms.

DIFFERENTIAL DIAGNOSIS:

1.   Granulomatous Secondary Syphillis
2.   Granulomatous Rosacea
3.   Sarcoidosis
4.   Tuberculoid Leprosy
5.  

SCROLL DOWN FOR ANSWER AND DISCUSSION.

CORRECT DIAGNOSIS:

Sarcoidosis

DISCUSSION:

Sarcoidosis is a chronic, multisystem disorder of unknown cause characterized in affected organs by an accumulation of T lymphocytes and mononuclear phagocytes, noncaseating epitheliod granulomas, and derangements of the normal tissue architecture. Although there is usually skin anergy and depressed cellular immune process in the blood, Sarcoidosis is characterized at the sites of disease by exaggerated T helper lymphocyte immune processes. All parts of the body can be affected, but the organ most frequently affected in the lung. Involvement of the skin, eye, and lymph nodes is also common. The disease is often acute or subacute and self limiting, but in many individuals it is chronic, waxing and waning over several years.

Cutaneous involvement in sarcoidosis may be classified as specific, which reveals granulomas on biopsy, or nonspecific, which is mainly reactive , such as erythema nodosum. The morphology of the lesions in sarcoidosis might include papuals, noduals, plaques, subcutaneous noduals, scar sarcoidosis, erythroderma, ulceration and verrucose, icthyosiform, hypomelanotic, psoriasiform, and alopecia. Sarcoid, like syphillis, is a great mimic of other skin diseases and should be considered in the differential diagnosis of many different skin disorders.

Sarcoidosis is a relatively common diseas affecting individuals of both sexes and almost all ages, races, and geographic locations. Females appear slightly more susceptable them males. In the United States , the majority of the patients are African American, whereas in Europe he disease affects mostly Caucasians

Histopathology

In most cases of sarcoidosis the epidermis is normal or slightly atrophic. Exceptions occur in variants of verrucous sarcoid where ther is acanthosis and hyperkeratoses; the latter is often seen in the ichthyosiform variant.

Granulomas, the hallmark of sarcoidosis, is seen in many forms throughout the dermis, with the location depending on the type of cutaneous lesion. Granulomas variably contain necrosis, although not often, or fibrosis, or granular material. Granulomas classicaly are surrounded by modest lymphocytic infiltrate at the periphery making the "naked granuloma".

Large and irregular shaped giant cells may be seen in the lesions of sarcoidosis, and variably contain asteroid and schaumann bodies. These bodies are also sen in other granulomatous disease processes such as tuberculosis. The asteroid bodies, more common that the schaumann bodies in sarcoidosis, may be formed by trapped colagen bundles and when stained show a star-shaped eosinophilic structure. Schaumann bodies contain calcium crystals, are round and oval in shape, and are found more often in sarcoidosis that in tuberculosis.

Diagnosis

For definitive diagnosis of sarcoidosis a biopsy is required, most commonly from the lung where the granulomatous process is most commonly found. In most cirrcumstances, the diagnosis of sarcoidosis is a combination of physical, histlogical, and radiographic findings, although without consistent blood findings, dianostic chest x-rays or scans(gallium 67), sarcoidosis is often confused with many other granulomatous disorders. Although not widley available, a helpful skin anergy test used is the Kviem-Siltzbach test.The test consists of an intradermal injection of a heatstabilized antigen of sarcoidosis which is biopsied 4-6 weeks later for evaluation of the development of a granuloma. The test yields sarcoid-like granulomas in 70 to 80% of individuals with sarcoidosis and has less than a 5% false positive reaction.

Angiotensin-converting enzyme (ACE) levels may be elevated in all granulomatous diseases, including sarcoidosis. An eleveated ACE level is suggestive but not diagnostic for granulomatous inflamation. Abnormal ACE level does not rule out sarcoidosis. If elevated, ACE levels may be used to monitor the activity of the disease.

TREATMENT:

Since most skin lesions of sarcoidosis are asymptomatic the major indication for threatment is cosmetic, ( except for disfiguring facial lesions). Systemic corticosteroids are virtually always beneficial an cutaneous sarcoidosis. Unfortunately , the dose required to control cutaneous disease may be too high to be ideal for long term use. Intralesional triamcinolone acetanide suspension 2.5mg/ml to 5.0mg/ml is very effective. For thinner lesions super potent topical steroids may be effective. Antimalarials chloraquine and hydroxychloroquine, and methotrexate have also shown partial or complete response.
Actual treatment for this patient

This patient was treated with topical 0.1% triamcinalone cream BID for 3 weeks and had resolution of erythema and a marked decrease in the number of lesions

REFERENCES:

1. Richard B. Odom, Andrews' Diseases of the Skin, 9th edition, W.B. Saunders Company, 2000

2. David Elder, Synopsis and Atlas of Lever's Histopathology of the Skin, Lippincott Williams & Wilkins, 1999

3. Anthony S. Fauci, Harrison's Principles of Internal Medicine, 14th edition, McGraw-Hill, 1998

Additional Comment:

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