Unusual Case of Granuloma Annulare To the Editor.\p=m-\Granulomaannulare is not an uncommon clinical cutaneous disease. A hereditary predisposition to this disease has been suspected because of reported cases in twins or siblings that have occurred almost simultaneously.1,2 In a comprehensive review of this disease, Wells and Smith note that less than 5% of patients have facial lesions, and that cases occurring in siblings are not common.1 The case reported here is distinctive because of the appearance of unilateral multiple lesions on the face and the positive family history of the disease. This healthy 10-year-old boy had first noticed a 2-cm oval lesion with an indurated border on the dorsal aspect of the right hand 1\m=1/2\years ago. Shortly thereafter, similar but smaller confluent lesions appeared on the right cheek, left forearm, and left hand. The lesions were oval patterns having a papular border and clear center. A biopsy specimen from the hand had been obtained one year

Fig 1— Many small annular lesions having papular border and smooth center on

right cheek.

Fig 2—Similar lesion

previously and was interpreted by the Armed Forces Institute of Pathology as granuloma annulare. The patient's older brother, current¬ ly 12 years of age, had had a similar eruption on the hands, trunk, and

lower extremities in 1967. These lesions had subsided spontaneously after 18 months. There is no other

pertinent family history. Another biopsy specimen of hand lesion from the patient

the was

interpreted as granuloma annulare.

Joseph W. Burnett, MD COL Taras Nowosiwsky, MC, USA Gary J. Calton, PhD M.H. Talezadeh, MD Baltimore

1. Wells

RS, Smith MA: The natural history of granuloma annulare. Br J Dermatol 75:199-205, 1963. 2. Spitzer R: Familial granuloma annulare. Dermatologica 123:38-41, 1961.

Multiple Mucosal Neuroma Syndrome To the Editor.\p=m-\The patient presented by Hurwitz in the transactions of the New England Dermatological Society1 may represent a case of the multiple mucosal neuroma syndrome (MMNS). Froboese2 first described this syndrome of multiple neuromas of the tongue, lips, and eyelids, associated with medullary carcinoma of the thyroid and pheochromocytoma. Gorlin et al3 later reviewed it. Sipple4 discussed the association between pheochromocytoma and thyroid carcinoma; he made no mention of mucosal lesions. Bolande5 distinguished between MMNS and Sipple syndrome, but grouped them together as possible neural crest disorders. Finally, the picture labeled "characteristic facies" is identical to those seen in MMNS

patients.

Walter Burgdorf, MD

Minneapolis

on

left hand.

1. Sipple syndrome, SOCIETY TRANSACTIONS. Arch Dermatol 110:139-140, 1974. 2. Froboese C: Das aus markhaltigen Nervenfasern bestehende ganglienzellenlose echte Neurom in Rankenform. Arch Pathol Anat 240:312\x=req-\ 327, 1923. 3. Gorlin RJ, Sedano HO, Vickers RA, et al: Multiple mucosal neuromas, pheochromocytoma, and medullary carcinoma of the thyroid. Cancer 22:293-299, 1968. 4. Sipple JH: The association of pheochromocytoma with carcinoma of the thyroid. Am J Med 31:163-166, 1961. 5. Bolande RP: The neurocristopathies. Human Pathol 5:409-429,1974.

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Reply To the Editor.\p=m-\Theassociation of

thyroid carcinoma and pheochromocy-

was first described in the American literature by Eisenberg in 1932; Sipple redirected attention to this disorder in 1961. It is unfortunate that in most early studies, family histories and other related findings were neither undertaken nor recognized. It has not been until recent years that mucosal neuromas, hyperparathyroidism, eye disorders, Cushing syndrome, von Recklinghausen disease, diabetes mellitus, and pigmentary disorders have been noted in association with medullary thyroid carcinoma and

toma

pheochromocytoma.

Terms associated with variations of the aforementioned constellation of findings include the Sipple syndrome (medullary thyroid carcinoma and pheochromocytoma), multiple endocrine neoplasia type II (medullary

thyroid carcinoma, pheochromocytoma, Cushing disease, and multiple mucosal neuromas), and the medullary thyroid carcinoma syndrome. The multiple mucosal neuroma syndrome (MMNS) includes neuromas of the lips, tongue, eyelids, and intestinal mucosa (with or without medullary thyroid carcinoma, depending upon the author describing the condition).

It now appears that these syndromes are pathogenetically related conditions that, within a given indi¬ vidual, may occur singularly or in multiple combinations. Manifesta¬ tions are thought to represent a regu¬ latory defect in the differentiation of chromaffin tissue, the elements of which probably originate from cells that migrate from the neural crest. Until now, the labeling of these syndromes has been dependent on the various combinations and permuta¬ tions that appear in the patient and/ or his family. An eponym attached to each new constellation of findings may add confusion to an already

complex terminology. Perhaps the time has come for these interrelated disorders to be desig¬ nated simply. I propose that they be referred to in the

same manner as

the

lipoproteinemias and the mucopolysaccharidoses; that is, to be termed "neural crest syndrome type 1, type 2, type 3, or type 4," or to be labeled as variations of the medullary thyroid carcinoma syndrome. Sidney Hurwitz, MD New Haven, Conn