Calvarial sarcoid mimicking metastatic diseaseBelin Fred Bodie, M.D., Sonia M. Kheir, M.D., and Emily F. Omura, M.D.Birmingham, AL
A 61-year-old white woman presented with localized cutaneousBoeck's sarcoid and multiple lytic skull lesions mimicking metastaticcarcinoma. Complete workup revealed no neoplastic process; biopsyof the skull lesions showed noncaseating granulomas consistent withsarcoid. Although rare, calvarial sarcoid can occur. (J A" ACADDER.\IATOL 3:401-405, 1980.)
Sarcoid is a well-known systemic granulomatous disease of unknown etiology. It can involveany organ system of the body including theskeletal system. Although sarcoid has been reported to involve most bones, it primarily affectsthe small bones of the hands and feet. This articledescribes a patient who developed multipleasymptomatic radiographic osteolytic lesions dueto sarcoid that mimicked a metastatic malignancy.
CASE REPORT
The patient was a 61-year-old white woman in anormal state of health until 3 years prior to admission,when she noted a persistent, slightly raised, pruriticplaque on her left shin. The leg lesions continued toenlarge gradually. Two and one-half years later, thepatient noted smaller but similar lesions developing onher forehead. Two years prior to admission, this ladyexperienced intermittent spells of "dizziness" precipitated by position changes. In the following 18 monthsshe developed several severe episodes of vertigo withnausea and vomiting. When she did not improve withdimenhydrinate (Dramamine) and hydroxyzine pamoate(Vistaril), she was admitted to a local hospital andfound to have multiple lytic skull lesions (2 to 30 mm indiameter), thought to be metastatic carcinoma. A physical examination and an extensive workup were completely normal except for the skin lesions, a diffuselyenlarged thyroid gland, and the abnormal skull x-ray.Her thyroid function tests were normal at this time. For
From the Departments of Dermatology and Pathology, The Univer-sity of Alabama in Birmingham Medical Center.
Reprint requests to: Dr. Belin Fred Bodie, 4300 Old Shell Road,Suite C, Mobile, AL 36608.
the next several months, the patient experienced occasional spells of weakness and lethargy but no anorexiaor weight loss. She was admitted to the University ofAlabama in Birmingham Medical Center for furtherevaluation.
On admission she denied any history of shortness ofbreath, hemoptysis, or exposure to known tuberculosisor beryllium. There was no history of fever, chills, oranorexia. The physical examination was normal exceptfor diffuse thyroid gland enlargement and isolated skinlesions involving the forehead, left arm, and left pretibial area, characterized as I-cm yellow-brown,slightly raised plaques with mildly depressed centers.
Normal laboratory studies included: complete bloodcount with differential, platelet count, urinalysis, electrolytes, glucose, blood urea nitrogen, creatinine,serum glutamic oxaloacetic transaminase (SOOT),creatine phosphokinase (CPK), gamma glutamyl transpeptidase, lactic dehydrogenase (LDH), total bilirubin,plasma thromboplastin time, and protime. Rapidplasma reagin was nonreactive. Serum calcium was 9.5mg/dl (normal, 8.5-10.5 mg/dl), phosphorus was 3.0mg/dl (normal, 3.5 mg/dl or I!1ore), total protein was7. I gm/dl (normal, 6.4-8.4 gm/dl), and albumin was3.82 gm/dl (normal, 3.5 gm/dl or more). Serum immunoelectrophoresis revealed a normal configuration,and urine immunoelectrophoresis demonstrated no lightchains.
The chest x-ray showed no evidence of active disease, but a tracheal deviation to the right was notedprior to its entry into the thorax; specifically there wasno hilar adenopathy. A thyroid scan was subsequentlyconsistent with multinodular goiter, explaining thecause of the patient's deviated trachea. An intravenouspyelogram was normal. Skull series showed "multiple
fairly well circumscribed lytic lesions throughout theskull which range in size from 4 mm to 4' em... (Fig.I). These appeared to arise from the diploic space andwere interpreted as probable metastatic carcinoma. Atechnetium 99m MDP bone scan showed increased activity involving the skull and ninth posterior rib, compatible with metastatic disease. A bone marrow biopsywas normal. A skin biopsy showed noncaseating granulomas throughout the dermis, extending into the subcutaneous fat (Fig. 2). These were discrete and consisted of large epithelioid cells; several multinucleatedgiant cells were present. Occasional asteroid bodieswere seen. The overlying epidermis showed no signs ofalteration, such as pseudoepitheliomatous hyperplasiausually encountered with infectious agents. Examination with polarized light for foreign bodies and stainsfor fungi and acid-fast bacteria were negative. Theskull biopsy showed similar granulomas extensivelyinfiltrating between the bony trabeculae (Fig. 3).
A tuberculin skin test was negative at 48 hours. Cultures of bone marrow and skull biopsy material werenegative for acid-fast bacilli and deep fungi. Pulmonaryfunction tests were within normal limits. Ophthalmologic examination of the patient's eyes demonstratedno evidence of sarcoid uveitis or retinitis. Kveim testwas not done. The patient was discharged with thediagnosis of sarcoid.
DISCUSSION
Since 1904, when Kreibich I first recognizeddestructive lesions in the phalanges of a patient
with lUpus pernio, the entity of skeletal sarcoid hasbecome well accepted. Mayock et al z reviewednine series of patients with sarcoid and found thatbetween 5.3% and 26% of the patients experienced bone changes.
Skeletal sarcoid usually is seen to occur in theterminal phalanges of the hands and feet. Onlyrarely does this disease process involve the calvarium, vertebrae, long bones, and ribs. We areaware of only twelve previously documented patients with calvarial sarcoid.:l- 11 Nearly all patients had multiple granulomatous lesions of theskull, and many were asymptomatic, as was ourpatient.
It is of interest to note that although Boeck'ssarcoid is more common in blacks, Neville et aPIfound twenty of twenty-four patients with osseoussarcoid to be Caucasian. Of those patients withcalvarial sarcoid whose race was documented (including the patient presented in this article), six ofseven were Caucasian. It is also noteworthy thatskeletal sarcoid is found more frequently in thosepatients with cutaneous sarcoid. 12
•13 Because thetechnetium 99m MDP bone scan showed positivefindings in the areas of the sarcoid granuloma ofthe skull, one can assume that skeletal granulomamay be localized using this technic.
The differential diagnosis of single or multiplelytic areas on skull x-rays includes: tuberculous
granuloma, eosinophilic granuloma, sarcoidgranuloma, deep fungal granuloma, other granuloma, metastatic disease, myeloma, meningioma,and fibrous dysplasia.
We present this patient to encourage the consideration of sarcoid as a possible etiology for lyticskull defects. In addition, observing such defects
on a person's x-ray should encourage the physician to examine the patient closely, looking forother changes suggestive of sarcoid. Should anyskin lesions be found, a skin biopsy may help todetermine the final diagnosis.We express our appreciation to Ms. Daphne Chamberlin for her assistance in preparing this paper.
404 Bodie el alJournal of the
American Academy ofDermatology
fig. 3. Skull biopsy: noncaseating granulomas infiltrating between the bony trabeculae.(Hematoxylin-eosin stain; x93.)
REFERENCESI. Kreibich K: Ober Lupus pernio. Arch Dermatol Syph
