71/80. skin sympathetic nerve activity in acquired idiopathic generalized anhidrosis. We recorded skin sympathetic nerve activity (SSNA) microneurographically from the right tibial nerve of a patient with acquired idiopathic generalized anhidrosis (AIGA). The patient did not show any spontaneous sweating or pilocarpine- and nicotine-induced sweat response. Histopathologic examination showed degenerated eccrine glands associated with surrounding inflammatory cellular infiltration. Electrical nerve stimulation produced a two-peak pattern of SSNA reflex discharge representing sudomotor and vasoconstrictor components. The frequency of spontaneous SSNA bursts (burst rate), presumably of a sudomotor nature, at the ambient temperature of 25 degrees C was significantly higher than in a healthy control subject and was further increased at a temperature of over 38 degrees C. Thus, sudomotor sympathetic nerve activity is well preserved or even increased in AIGA. We conclude that anhidrosis of AIGA results from the generalized sweat gland dysfunction rather than decreased sympathetic outflow to the skin. ( info) |
72/80. The hybrid epidermoid and apocrine cyst. A combination of apocrine hidrocystoma and epidermal inclusion cyst. We describe four cases of unusual superficial cutaneous cysts that have a lining epithelium composed of apocrine cells immediately adjacent to keratinizing squamous epithelium with an intact granular layer. Two of the cysts were on the nipple of the breast and two of the cysts were on the face. The lumina of all four cysts contained keratin. Immunoperoxidase staining for carcinoembryonic antigen in the facial cysts showed positive staining within the apocrine cells of the lining epithelium in one case. The pathogenesis of these lesions is uncertain. We suggest that these unusual cysts be called hybrid epidermoid and apocrine cysts. ( info) |
73/80. Porokeratotic eccrine ostial and dermal duct nevus of late onset: more frequent than previously suggested? We describe the case of a 10-year-old boy with a porokeratotic eccrine ostial and dermal duct nevus (PEODDN) of late onset. The patient had an 8-year history of multiple keratotic papules on the dorsal surface, and multiple yellowish pitting lesions on the plantar surface of the right foot. light-microscopic studies of both lesions showed multiple cornoid lamella-like parakeratotic columns, which exclusively arose over eccrine sweat ducts in which the acrosyringium was dilated. Although PEODDN is considered to be a congenital hamartoma of eccrine origin, a review of the literature showed us that the frequency of a late-onset variant may be as high as 26%. ( info) |
| hidradenitis suppurativa is a chronic, recurrent, suppurative inflammation of apocrine glands. morbidity is high and serious complications may occur. A technique of surgical treatment utilizing an electrosurgical unit to provide cutting current is described. This allows rapid unroofing and cauterization of extensive cysts and sinus tracts. ( info) |
| pseudohypoaldosteronism is an uncommon disorder characterized by urinary sodium wasting and is attributed to a defect in distal renal tubular sodium handling with failure to respond to endogenous aldosterone. Sweat electrolyte values in other reported patients, when measured, have been normal. A 3.5-year-old girl developed repeated episodes of dehydration, hyponatremia, and hyperkalemia during the first 19 months of life. serum sodium was as low as 113 mEq/liter and potassium as high as 11.1 mEq/liter. Her plasma and urinary aldosterone levels were persistently elevated (Figs. 1-4). Unlike patients with classic pseudohypoaldosteronism she demonstrated no urinary sodium wasting (Figs. 2 and 3). During episodes of hyponatremia and reduced sodium intake her urinary sodium was less than 5 mEq/liter. In addition, her sweat sodium concentration was consistently above 125 mEq/liter and salivary sodium concentration above 58 mEq/liter. Her chest x-ray, 72-hr fecal fat excretion, serum and urinary pancreatic amylase (amy-2) were normal, providing no evidence for cystic fibrosis. It is proposed that this patient represents a new variant of pseudohypoaldosteronism with excessive loss of sodium from the sweat and salivary glands instead of the kidneys. ( info) |
| Two overweight male patients developed acanthosis nigrican of the axillae. Seven to nine months later, the patients developed hidradenitis suppurativa deep within the areas of acanthosis nigricans. On examination of biopsy specimens, the hyperkeratosis of acanthosis nigricans was found to extend into the follicles. In previous reports, fox-fordyce disease and pityriasis rubra pilaris have preceded the onset of hidradenitis suppurativa. ( info) |
77/80. Agminate and plaque-type blue nevus combined with lentigo, associated with follicular cyst and eccrine changes: a variant of speckled lentiginous nevus. Agminate and plaque-type blue nevi are rarely described. We report the occurrence of such a type of blue nevus associated with eccrine changes and a follicular cyst all arising on a macular brown background in a 38-year-old man. The patient presented numerous blue papules and a plaque, overlapping a light tan patch present since birth, on his left thigh. In addition, within the plaque, 3 papules, discharging at intervals a serous fluid, were present. Since the lesion hardened and enlarged, it was surgically excised. Histologic findings revealed a lentigo pattern of the epidermis, corresponding to the light tan macular background and a plaque-type blue nevus, and areas of eccrine ductal proliferations with a ruptured follicular cyst. The association of agminate and plaque-type blue nevus, arising on a light brown patch of lentigo, might represent a variant of speckled lentiginous nevus. Eccrine proliferations may be reactive in nature or represent a more complex hamartomatous lesion. The possibility of malignant transformation and the recent enlargement of the lesion caused by the ruptured follicular cyst convinced us to carry out a wide surgical excision. ( info) |
78/80. Porokeratotic eccrine ostial and dermal duct naevus with dermatomal trunk involvement: literature review and report on the efficacy of laser treatment. Porokeratotic eccrine ostial and dermal duct naevus (PEODDN) is a rare, benign hamartomatous malformation involving the eccrine sweat duct. The existence of filiform keratinous plugs that represent cornoid lamellae overlying dilated infundibula of eccrine ducts is a distinctive feature and the presence of associated abnormal dermal ducts is frequent. We report a patient with PEODDN who exhibited lesions on the left side of her chest. Cases of PEODDN reported in the literature are reviewed. Our experience in treating this patient with ultrapulsed carbon dioxide laser is also presented. ( info) |
79/80. Multiple follicular hamartomas with sweat gland and sebaceous differentiation, vermiculate atrophoderma, milia, hypotrichosis, and late development of multiple basal cell carcinomas. We describe a 46-year-old woman with multiple symmetrical papulocystic lesions on the face, neck, chest, back, and upper arms since childhood. Vermiculate atrophoderma, entropion, and progressive loss of eyelashes was also noted. Since the age of 30, 12 basal cell carcinomas have developed on the face. Histopathologic examination revealed multiple anastomosing nests and strands that arose from the lower part of a hair follicle in early lesions. In more advanced lesions, multiple keratinizing microcysts within a moderately sclerotic stroma were noted in the upper and mid dermis. In some biopsy specimens, focal areas showing sweat gland (ductal) and sebaceous differentiation were also observed. Basal cell carcinomas developed from the basaloid component of the hamartomatous proliferation. ( info) |
| A new syndrome, consisting of natal or defective teeth, or both, steatocystomas of the skin, and epidermal cysts of the scalp, is described in several generations. One member of the family had eruptive molars. male-to-male transmission suggests autosomal dominant inheritance. This syndrome should be separated from pachyonychia congenita I and II. ( info) |