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BACKGROUND AND DESIGN: Trichoepithelioma is a benign skin tumor with follicular differentiation, which sometimes is difficult to distinguish clinically and histologically from basal cell carcinoma. One of the most helpful differences is the histologic appearance of the stroma. CD34 is an antigen known to stain the spindle-shaped cells located around the middle portion of normal hair follicles. We have stained formalin-fixed, paraffin-embedded sections of 16 trichoepitheliomas and 19 basal cell carcinomas for CD34 (anti-HPCA-1, Becton Dickinson, San Jose, Calif) to detect differences in the staining pattern and to facilitate discrimination of these two types of tumors. RESULTS: The spindle-shaped cells surrounding the islands of trichoepithelioma cells were focally strongly positive for CD34. In all basal cell carcinomas, the spindle-shaped cells surrounding the nests of tumor cells were negative; in these areas only the blood vessels were positive with this antibody. CONCLUSIONS: CD34 staining pattern differentiates between trichoepithelioma and basal cell carcinoma. CD34 stain may be helpful in distinguishing between these two tumors on small punch biopsies or in difficult diagnostic cases.(Arch Dermatol. 1994;130:589-592)

• BACKGROUND.— Restrictive dermopathy is a lethal genetic disorder consisting of abnormally tight skin, generalized joint contractures, distinctive facies, and pulmonary hypoplasia. Autosomal recessive inheritance has been suggested based on multiply affected siblings and some reports of parental consanguinity. This article describes two siblings with the restrictive dermopathy syndrome and reviews previously reported cases. OBSERVATIONS.— Eight other cases have been reported in the literature as restrictive dermopathy. These cases have shared striking similarities in their clinical histories and phenotypes. The skin in these infants has been described as rigid and tense, with skin biopsy specimens showing a thick epidermis, thin dermis, abnormally arranged collagen bundles, and poorly developed appendages. Other prominent features are flexion contractures and craniofacial and pulmonary abnormalities. The genetic and/or developmental defects leading to the restrictive dermopathy syndrome are presently not known. CONCLUSIONS.— The restrictive dermopathy syndrome is distinct and is easily differentiated from other congenital diseases such as the icthyoses and also from the clinical conditions of sclerema neonatorum and subcutaneous fat necrosis of the newborn. Recognition of this syndrome is important for determining the prognosis of affected infants and for recommending genetic counseling to affected families.(Arch Dermatol. 1992;128:228-231)

• Benign cutaneous polyarteritis nodosa has been described as having a benign course in contrast to that of systemic classic polyarteritis nodosa. We tested the hypothesis that this histologic distinction is false by reviewing nine consecutive cases with the histologic diagnosis of benign cutaneous polyarteritis nodosa. Our study revealed that on follow-up, seven (78%) of nine cases had evidence of involvement of at least one organ other than the skin, with the kidney being the organ most commonly involved. Four (44%) of nine patients had serologic evidence of hepatitis B infection, one had cryoglobulinemia, and one had polyclonal hypergammaglobulinemia associated with acquired immunodeficiency syndrome. We conclude that benign cutaneous polyarteritis nodosa is not necessarily benign and is closely related to systemic polyarteritis nodosa.(Arch Dermatol. 1991;127:1520-1523)

• Neurofibromas, schwannomas, and neurotized melanocytic nevi may closely resemble one another at the light microscopic level. We studied 10 neurofibromas, 10 schwannomas, and 10 partially neurotized melanocytic nevi immunohistochemically using an antibody directed against factor XIIIa to determine if this antibody might provide a useful method of differentiating these lesions. The cases were also stained with S100 protein. All of the neurofibromas stained intensely for factor XIIIa. The proportion of cells staining within the tumors varied from 30% to 70%. In contrast, none of the schwannomas and neurotized nevi studied demonstrated staining of tumor cells with this antibody. S100 protein was expressed by 100% of neurofibromas, schwannomas, and melanocytic nevi. Our findings suggest that factor XIIIa may provide a reliable and practical means of differentiating cutaneous neurofibromas from neurotized nevi and cutaneous schwannomas. Distinguishing between these different tumor types may be important in some clinical situations, particularly with respect to rendering a diagnosis of von Recklinghausen's neurofibromatosis. The differences in the immunohistochemical profiles of neurofibromas and neurotized nevi support the concept that these tumors are histogenetically distinct, despite their similar histologic appearances.(Arch Dermatol. 1990;126:472-476)

• Low-fluence carbon dioxide (CO2) laser irradiation of skin has previously been shown to induce damage limited primarily to the epidermis. To evaluate whether this technique was therapeutically effective for pigmented epidermal lesions, ten lentigines caused by methoxsalen and ultraviolet light therapy were treated in one patient using the CO2 laser at fluences ranging from 3.0 to 7.7 J/cm2 for 0.1-s exposures with 4.5-mm spot size. Based on substantial clearing in seven of ten lesions treated, 146 solar lentigines were treated in five patients at fluences of 3.0, 3.7, or 4.4 J cm2. Biopsies were performed on a total of 30 lesions immediately and 24 hours, seven days, and six weeks after irradiation. Of 125 lesions followed up clinically for six weeks, 12 cleared completely, 81 lightened substantially, and 28 remained unchanged. Only two demonstrated atrophic change. Hyperpigmentation or hypopigmentation did not occur. All lesions that improved had been treated at 3.7 or 4.4 J/cm2. Immediate histologic injury consisted of vacuolar and spindly change and subsequent vesiculation limited to the basilar epidermis. Twenty-four hours later there was epidermal necrosis with regeneration, 0.1 mm of dermal basophilia and stromal condensation, and a mild inflammatory infiltrate. These alterations were dose-dependent, with near complete epidermal necrosis and superficial dermal involvement at the highest fluence, and only focal epidermal necrosis at the lowest. At seven days, epidermal regeneration was complete with traces of melanin remaining in keratinocytes. Melanophages first appeared at seven days and persisted at six weeks, by which time the inflammatory infiltrate had cleared. No lentiginous proliferation was evident and epidermal pigmentation had become normal. Low-fluence CO2 laser irradiation is an effective means of damaging the epidermis with only minimal dermal change. This mode of therapy is an effective way to lighten the pigmentation of lentigines without substantial scarring.(Arch Dermatol 1988;124:1219-1224)

• Port-wine stains result from a progressive ectasia of the cutaneous superficial vascular plexus. One hypothesis for the pathogenesis of this lesion is an abnormal neural regulation of blood flow. Biopsy specimens of 11 port-wine stains, seven hemangiomas, and 17 benign lesions were stained for S100 protein using immunoperoxidase techniques. All specimens were of facial biopsies or excisions and were evaluated for vessels per square millimeter, nerves per square millimeter, vesselto-nerve ratio, and frequency of vessels coursing within 0.03 mm of nerves. These variables were evaluated in the superficial 0.8 mm of dermis, a zone that includes almost all abnormal port-wine-stain vessels. Controls showed 18.3 ± 2.8 vessels/sq mm (±SD), 21.1 ± 9.2 nerves/sq mm, 0.9 ± 0.3 vessels to nerves, and 75% ± 11% of vessels coursing within 0.03 mm of nerves, values that did not alter with age. Port-wine stains had a significant decrease in nerve density and increase in vessel-to-nerve ratio when compared with normal skin; only 17% ± 3% of vessels were associated with nerves in port-wine stains. These findings document a deficit in the number of perivascular nerves in port-wine stains and raise the possibility that a lack of neural modulation of vascular flow may be involved in the pathogenesis of port-wine stains.(Arch Dermatol 1986;122:177-179)

† BACKGROUND.— Blue-gray pigmentation of the skin can be elicited by several medications. We report the first case (to our knowledge) of desipramine-induced photosensitive blue-gray pigmentation. OBSERVATIONS.— Diffuse blue-gray pigmentation on sun-exposed surfaces was noted in a healthy 48-year-old woman who had been taking desipramine hydrochloride for 8 years. Ultrastructural studies demonstrated the presence of melanin and homogeneous electron-dense material in the dermis. CONCLUSIONS.— We conclude that tricyclic antidepressant agents represent another class of medications responsible for blue-gray cutaneous pigmentation.(Arch Dermatol. 1993;129:474-476)

TO THE EDITOR.— Several tumors and tumorlike lesions of fibrohistiocytic origin occur in the skin.1,2 Herein we describe a distinctive tumor of fibrohistiocytic origin that has not, to our knowledge, been previously reported. REPORT OF CASES.—CASE 1.— A 38-year-old Hispanic woman presented to the New York Hospital-Cornell Medical Center, New York, NY, dermatology clinic with a 2 ½-year history of a slowly enlarging, asymptomatic lesion of her right cheek (Fig 1). There was no history of trauma preceding the appearance of the lesion. On examination the tumor was approximately 2 cm in diameter and protruded 1.8 cm from the skin surface. The epidermis covering the tumor was hyperpigmented and a hyperkeratotic scale was present at the apex. By palpation the tumor was firm and appeared well demarcated. Examination of the oral cavity revealed a normal-appearing buccal mucosa. Examination of the patient was otherwise unremarkable. In particular, there

TO THE EDITOR.— Ferguson Smith1 did not realize that he had discovered an autosomal-dominant trait when he reported a single case of multiple self-healing squamous carcinomas (MSHSC) in 1934.2 It is now recognized that both familial and sporadic types of MSHSC exist. Both entities are now included in the designation of multiple Ferguson-Smith keratoacanthomas. The MSHSC are occasionally known for an extremely mutilating course and fatal outcome. The patient initially described by Ferguson Smith was again cited 14 years later for extensive nasal destruction that required a prosthesis.3 Charteris4 described a patient with MSHSC who died as a result of an extensive recurrent anal lesion. We report a patient with sporadic MSHSC who experienced extensive recurrent perineural invasion of the right supratrochlear nerve. This observation suggests that perineural invasion by keratoacanthoma should not be regarded as an entirely benign condition. REPORT OF A CASE.— A 44-year-old