Subungual basal cell carcinoma presenting as longitudinal melanonychia

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ylococcal colonization of the patient's own skin and subsequent contamination of the wound." This case serves as an important reminder that any surgical procedure, including sihaple skin excision, can be complicated by life-threatening toxic shock syndrome. As illustrated by our patient, it is also important to recognize that the inciting wound infection can occur in the absence of erythema and purulence. Patients who have undergone recent surgical procedures who then exhibit possible signs of toxic shock syndrome need to have even normal-appearing wounds reopened for diagnostic as well as therapeutic purposes. Physicians performing cutaneous surgery should be familiar with the signs and symptoms of toxic shock syndrome because early recognition and intervention can prove to be lifesaving. REFERENCES 1. Reingold AL, Hargrett NT, Shands KN, et al: Toxic shock syndrome surveillance in the United States, 19801981: Ann Intern Med 96:875-880, 1982.

Toxic shock syndrome

2. Reingold AL, Hargrett NT, Dan BB, et al: Nonmenstrual toxic shock syndrome. Ann Intern Med 96:871-874, 1982. 3. Barlett P, Reingold AL, Graham DR, et al: Toxic shock syndrome associated with surgical wound infections. JAMA 247:1448-1450, 1982. 4. Morrison VA, Oldfield ED: Postoperative toxic shock syndrome. Arch Surg 118:791-794, 1983. 5. Bach MC: Dermatologic signs in toxic shock syndrome: Clues to diagnosis. J AM ACAD DERMATOL8:343-347, 1983. 6. Doman KJ, Thompson DM, Conn AR, Wittmann BK: Toxic shock syndrome in the postoperative patient. Surg Gynecol Obstet 154:65-68, 1982. 7. Aganaba T, Evans RP, O'Neill P: Toxic shock syndrome after orchidectomy. Br Med J 286:685, 1983. 8. Portnoy D, Hinchey EJ, Marcus-Jones OW, Richards GK: Postoperative toxic shock syndrome in a man. Can Med Assoc J 126:815-816, 1982. 9. Altemeier WA, Lewis SA, Bjomson HS, et al: Staphylococcus in toxic shock syndrome and other surgical infections: Development of new bacteriophages. Arch Surg 118:281-284, 1983. 10. Davis JP, Chesney PJ, Want PJ, LaVenture M: Toxic shock syndrome. N Engl J Med 303:1429-1435, 1980. 11. Mortimer EA: Possible mechanisms for vaginal infection with Staphylococcus aureus. Ann Intern Med 96:934936, 1982.

Subungual basal cell carcinoma presenting as longitudinal melanonychia Robert I. Rudolph, M.D.* Wyomissing, PA Subungual basal cell cancers are extremely rare, and a case is presented herein. This cancer had as its only manifestation the unique presence of a deeply pigmented streak in the nail for many years. Longitudinal melanonychia can signal baleful or banal conditions, and basal cell cancer must now be added to this list. (J AM ACriD DERMATOI. 1987;16:229-33.)

Basal cell cancers are by far the most common skin malignancy, usually occurring in actinically damaged skin. These cancers can, however, deFrom the Department of Dermatology,Hospital of the Universityof Pennsylvania. No reprints available. *Dr. Rudolph is in private practice.

velop in unlikely sites, such as the palm, the axilla, and even the nipple.~'2 An unusual location for a basal cell cancer is the subungual area, and a case of a basal cell cancer in this rare area is presented herein. The case is clinically even more unusual in that a pigmented streak in the nail was the only indication of the underlying malignancy. This longitudinal melanonychia in association with a sub229

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Rudolph

Table I. Causes of longitudinal melanonychia*

Fig. 1. Deeply pigmented streak of the left thumbnail.

ungual basal cell cancer is unique, and basal cell carcinoma must n o w be added to the list of diseases that can present as a pigmented nail. CASE REPORT A 59-year-old white woman sought evaluation because a dark streak had been present in her left thumbnail for over 15 years (Fig. 1). At no time was there any involvement of the periungual areas, and the nail had never become soft or crumbly. There was only a little discomfort on deep pressure, and bleeding had never occurred. The thumb had never been excessively traumatized, nor had the patient ever had any type of irradiation to the digit. She had received oral griseofulvin for several months because of a diagnosis of "fungus of the nail," with no help. The nail had also been removed on several occasions, but the pigmented streak returned as the nail regrew. Examination showed the nail itself to be slightly ridged, but it was otherwise intact except for a 3-mmwide brown streak extending along the entire length of the nail. The cuticular and periungual areas were norreal. No lymph nodes were felt in the epitrochlear or axillary areas. The other fingernails were entirely normal. A radiograph of the thumb was normal, showing no bony abnormalities. A longitudinal biopsy of the entire nail bed and matrix area was performed, and pigment was visible grossly in the nail matrix and proximal nail bed region at the time of biopsy. Pathologic examination revealed nests of basophilic cells with the typical palisading morphologic picture of a basal cell carcinoma protruding from the surface epithelium and "hanging down" into the subadjacent tissue (Figs. 2 to 4). These basaloid ceils were associated with pigment. A stain for S-100

Addison's disease Adrenalectomy for Cushing's disease Bacterial infection Drugs Fungal infection Hematoma hemosiderosis Idiopathic cause Irradiation Laugier-Hunziker's syndrome Lentigo Malignant melanoma Malnutrition Melanotic hyperplasia (benign or atypical) Metastatic melanoma Nevocytic nevus Peutz-Jeghers-Touraine syndrome Photochemotherapy Porphyria cutanea tarda Pregnancy Radiodermatitis Splinter hemorrhages Trauma Vitamin B12 deficiency *ModifiedfromBaranR, DawberRPR, editors:Diseasesof the nails and theirmanagement.Oxford,England, 1984, BlackwellScientific Publications, Ltd.

protein was negative, indicating no melanoma or cells of neural crest derivation. DISCUSSION Subungual basal cell cancer is exceedingly uncommon, and only 10 cases have been reported since the first description by Eisenklam in 1931. 3-9In all cases the lesions have occurred on the fingers, except for one lesion on a toe. The usual presentation is as a chronic paronychia or periungual eczematous process often associated with ulceration, granulation tissue, and pain. The condition often persists for m a n y years, resisting various oral and topical therapies. In several cases the nail itself had been removed repeatedly, only to have the condition recur. After biopsy, definitive therapies have ranged from radium treatments to amputation of the affected digit and, most recently, Mohs chemosurgery. This rare tumor is unlike others in that at no time did the underlying tumor express itself'as a

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Subungual basal cell carcinoma

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o

Fig. 2. Basal cell cancer of nail bed and matrix area. (Hematoxylin-eosin stain; × 40.)

Fig. 3. Basal cell cancer from subungual area. (Hematoxylin-eosin stain; × 160.) chronic paronychia, nonhealing ulceration, or unresponsive eczematous process. It is not surprising that a slowly growing basal cell carcinoma could be present in the subungual area for years without spread or surrounding destruction, since clinicians frequently encounter slowly growing basal cell carcinomas that have been present for many years on the skin. The presence of longitudinal melanonychia, on the other hand, appears to be totally unique, and certainly none of the other reported cases had any

pigmentary changes described. Pigmented basal cell cancers are, of course, very common, and indeed the biopsy showed much pigment within the basal cell carcinoma of the nail bed and matrix. The abundance of pigment-producing cells and of pigment in this basal cell carcinoma was reflected in the nail as nail streaking. The negative S-IO0 protein stain indicated that the pigment-producing tumor was not a melanoma. The presence of acquired longitudinal melanonychia in a white patient is historically a cause

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Fig. 4. Close-up view showing basaloid cells of basal cell carcinoma. (Hematoxylin-eosin

stain; x 400.)

for great and often justified concern, since it is often a presenting sign of a subungual acral lentiginous malignant melanoma. A list of other conditions that can also produce longitudinal melanonychia is, however, relatively long and constantly expanding m~ (Table I). Subungual basal cell carcinoma must now also be added to this list and must be considered in the differential diagnosis of pigmented streaks of the nail. Because the variety of lesions and conditions that can produce longitudinal melanonychia is large and ranges from the completely banal to the awesomely malignant, it seems reasonable and prudent to recommend that any recent or suspicious streak in a nail--especially in a white patientmhave prompt and adequate biopsy, lz Radiographs of the affected digit can also be a useful diagnostic adjunct, since detectable bone changes will obviously mandate a different and much more aggressive surgical approach to the lesion. The major concern in this patient was to determine whether a subungual malignant melanoma was present, and the finding of a basal cell cancer was totally unexpected. Adequate biopsy saved this patient from the fate of others with subungual basal cell carcinoma--amputation of the distal digit--and permitted Mohs chemosurgery to be performed, eradicating the malignancy and yet al-

lowing the digit to be functional as well as cosmetically acceptable. 9'~3 The importance of an adequate nail biopsy is again demonstrated by this case, and it is therefore urged that when the clinician is presented with a pigmented streak of the nail, an adequate biopsy be performed early so that pathologic determination of the diagnosis can be obtained and unnecessary disfigurement and loss of function prevented. REFERENCES

1. Bruce S, Tschen JA, Goldberg LH: Basal cell cancer of the nipple. J Dermatol Surg Oncol 11:424-425, 1985. 2. RobinsP, RabinovitzHS, Rigel D: Basal cell carcinomas on covered or unusual sites of the body. J Dermatol Surg Oncol 7:803-806, 1981. 3. Eisenklam D: 0ber subunguale Tumoren. Wien Klin Wochenschr 44:1192-1193, 1931. 4. Levine J, Lisa JR: Primary carcinoma of the nail. Arch Surg 38:107-112, 1939. 5. Ashby BS: Primary carcinoma of the nail-bed. Br J Surg 44:215-217, 1956-1957. 6. NelsonLM, HamiltonCF: Primary carcinoma of the nailbed. Arch Dermatol 101:63-67, 1970. 7. Alpert LI, Zak FG, Werthamer S: Subungual basal cell epithelioma. Arch Dermatol 106:599, 1972. (Letter to Editor.) 8. Hoffman S: Basal cell carcinoma of the nail-bed. Arch Dermatol 108:828, 1973. 9. MikhailGR: Subungual basal cell carcinoma. J Dermatol Surg Oncol 11:1222-1223, 1985. 10. Baran R, Dawber RPR, editors: Diseases of the nails and

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their management. Oxford, England, 1984, Blackwell Scientific Publications, Ltd., p. 439. 11. Baran R, Jancovici E, Sayag J, et al: Longitudinal melanonychia in lichen planus. Br J Dermatol 113:369, 1985. 12. Salasche SJ, Garland LD: Tumors of the nail, in Daniel

Subungual basal cell carcinoma

CR, editor: Symposium on the nail. Philadelphia, 1985,' W. B. Saunders Co., pp. 514-517. (Dermatologicclinics, vol. 3.) 13. Mikhail GR: Subungual epid-ermoid carcinoma. J AM ACADDERMATOL11:291-298, 1984.

Anhidrosis (hypohidrosis) in Sj6gren's syndrome Jerry Mitchell, M . D . , * John Greenspan, B.D.S., Ph.D.,** Troy Daniels, D.D.S.,** John P. Whitcher, M.D.,*** and Howard I. Maibach, M.D.* San Francisco, CA There has been a relative lack of literature on the association of hypohidrosis in Sj6gren's syndrome with any lesion having specific histologic findings. We looked at a recent case presentation of a 55-year-old man with complaints of dry mouth and dry eyes, becoming easily overheated in direct sunlight, and having difficulty in perspiring. Physical examination showed fissuring and atrophy of the tongue and angular cheilitis. A punch biopsy of the skin showed a moderate number of eccrine gland and ductal structures in the lower reticular dennis, each surrounded by a dense cellular infiltrate of plasma and lymphocytic cells. Our patient also had a markedly decreased sweating response to methacholine. In reviewing the literature as far back as 1951 and on the basis of findings in our present case study, we conclude that it seems probable that the severity of the skin disease is an important determining factor in predicting whether the sweat gland lesion does exist. (J AM ACAD DEaMATOL 1987;16:233-5.)

Since 1933, when Henrik Sjrgren, the Swedish ophthalmologist, described in detail the clinical and histologic components of the syndrome, Sjrgren's syndrome has remained a challenge to diagnose and effectively treat. In this review we will demonstrate the association of symptomatic hypohidrosis with Sj6gren's syndrome and parallel the histologic findings with those in the salivary gland. From the Departmentsof Dermatology,*Stomatology,**and Ophthalmology,***Universityof CaliforniaSchoolof Medicine. Reprint requests to: Dr. HowardI. Maibach, Departmentof Dermatology, Universityof CaliforniaHospital, San Francisco,CA 94143.

CASE REPORT A 55-year-old man was seen in the Sjrgren's syndrome clinic of the department of stomatology in April 1985. The patient complained of having had dry eyes for approximately 2 years and a dry mouth for 6 months. For the last 6 months he had experienced difficulty in perspiring and became easily overheated in direct sunlight. Other complaints were of dysphagia and "puffiness" under the jaw. Oral examination showed no salivary gland enlargement; there was evidence of erythema, fissuring, and papillary atrophy of the tongue, as well as right angular cheilitis. The stimulated parotid flow rate was reduced to approximately one tenth of the normal rate. Ophthalmologic examination revealed evidence of moderate clinical keratoconjuncti233