[Physiopathogenesis of functional hypertrichosis. I. Specific aspects of androgen metabolism in functional hypertrichosis and total physiopathogenesis].
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Hypertrichosis lanuginosa is a pathologic state characterized by an excessive, new growth of fine, fetal hair. Two cases of hypertrichosis languinosa with malignancy (lymphoma and uterine cancer) are presented and added to the 9 in the literature. Lymphoma and uterine cancer are previously unreported as associated with hypertrichosis osis lanuginosa. Review of the 11 cases of hypertrichosis lanuginosa revealed the following characteristics: females were predominant; none was below the 4th decade; all had advanced neoplastic disease; all malignancies except one were of epithelial origin; and there were no demonstrable endocrine abnormalities. Despite an attempt to find etiologic factors in our patients and in the literature, none could be elicited.
Prepubertal simple hypertrichosis is characterized by excessive growth of vellus hair in children without other signs of endocrinopathies. The aetiology is unknown and it is not clear if it is an abnormal entity or an extreme form of the normal range of hair growth. Sixteen girls (aged 7 +/- 1.9 years, mean +/- SD) with prepubertal simple hypertrichosis and, as controls, 12 normal age-matched girls were studied. All patients were in preadrenarchal age and in all patients an ACTH test was performed to exclude non-classical forms of congenital adrenal hyperplasia. Testosterone (T), androstenedione (A), dehydroepiandrosterone sulphate (DHA-S), dihydrotesterone (DHT), 3 alpha-androstanediol (3Ad) and its glucuronide (3AG), and sex hormone binding globulin (SHBG) were evaluated and free testosterone (FT) and T/SHBG ratio were calculated in all subjects. In all patients we found T, A and DHA-S plasma levels comparable to controls, excluding an increased glandular androgen secretion. No significant differences, compared to controls, were observed for SHBG, FT and T/SHBG ratio, suggesting a normal T bioavailability. DHT plasma levels were significantly increased with respect to controls (0.42 +/- 0.04 vs 0.11 +/- 0.03 nmol/l; P less than 0.002) whereas 3Ad and 3AG were comparable to controls. The very significant increase in DHT plasma levels, without a parallel increase in 3Ad and 3AG found in our cases with prepubertal simple hypertrichosis, is difficult to explain. The clinical and biochemical significance of the high DHT plasma values needs more investigation.
Hypertrichosis is a well-recognized adverse effect of therapy with either cyclosporine or minoxidil. We present a case of extreme hypertrichosis in an eight-year-old male liver transplant recipient who received concurrent cyclosporine and minoxidil therapy. A review of drug-induced hypertrichosis is presented.
We have studied 2 Egyptian sibs (the offspring of normal first cousins) with congenital cataract, hypertrichosis, mental retardation, and normal chromosomes. Review showed that the condition of our patients was not similar to any previously reported entity. POSSUM lists 84 syndromes with any of the above 3 main traits. Two disorders with cataract and mental retardation, Martsolf syndrome and Mollica-Pavone-Anterer syndrome, have overlapping manifestations and therefore are particularly differentiated from our cases. We suggest that the association of congenital cataract, hypertrichosis, and mental retardation observed in this report represents a new autosomal recessive syndrome.
A patient with hypertrichosis lanuginosa acquisita associated with pancreatic islet cell carcinoma, probably D cell type, is presented. The hypertrichosis antedated other evidence of malignancy.
Hypertrichosis lanuginosa (without gingival hyperplasia) is described in a mother and son; the latter also had photophobia, infantile genitalia, growth retardation, hypotension, low IQ and dental abnormalities (hyperdontia, permanence of deciduous and delayed eruption of permanent teeth). Both have normal dermatoglyphics. Some clinical findings are discussed. The presence of this syndrome in a mother and son supports an autosomal mode of inheritance (with variable expressivity). Hypertrichosis lanuginosa is a pure monomultidysplasia and may be classified with the tricho-odontic sub-group of the ectodermal dysplasias.
1 61-year-old man had osteosclerotic myeloma that was localized in the eleventh thoracic vertebral body and associated with sensorimotor polyneuropathy, skin hyperipigmentation, edema, hypertrichosis, gynecomastia, and white nails. Cases of osteosclerotic myeloma with and without polyneuropathy in the literature were reviewed with special reference to accompanying dermatologic and endocrinologic signs and synmptoms. We assume that the polyneuropathy, cutaneous hyperpigmentation, edema, hypertrichosis, gynecomastia, and white nails are causally related to each other and are a remote effect of osteosclerotic myeloma. Quantitative histologic analysis of two sural nerves biopsied within 2 years of each other during the course of the disease indicated that both large and small myelinated fibers degenerated progressively, with relative preservation of unmyelinated fibers.
A 46-year-old nulligravida complained of the recent development of an erythematous skin eruption and fine blond hair over her face. These complaints appeared to be symptoms of acquired hypertrichosis lanuginosa, which may be associated with malignancy. The patient was found to have an endometrial adenocarcinoma with nodal metastases and was treated with hysterectomy and irradiation. Eighteen months later there was no evidence of the cancer, and the lanugo hairs had vanished. This is the first known instance of hypertrichosis lanuginosa associated with a gynecologic cancer and the first ever observed in which the lanugo hairs disappeared after cancer therapy.
Hypertrichosis lanuginosa acquisita is regarded as an obligatory cutaneous paraneoplasm and is defined as the sudden and excessive appearance of lanugo hairs on the entire integument associated with malignant neoplasm of internal organs. We observed such a case of hypertrichosis in a 30-year-old man with a long history of ulcerative colitis who developed carcinoma of the caecum with lymph node metastasis.
A case of gingival fibromatosis with hypertrichosis is reported in a 10 year old girl. The condition is a syndrome and presents as a triad of gingival fibromatosis, hypertrichosis and mental retardation. Following surgical management, no recurrence was seen in the immediate post-surgical period. Even if recurrence occurs excision is recommended as the psychological benefits resulting from cosmetic improvement outweigh the danger of recurrence.
The development of a malignant tumor, as well as hypertrichosis lanuginosa as a paraneoplasic syndrome, in a patient afflicted with scleroderma is a very rare event. We present the clinical history of a patient afflicted with scleroderma, non-differentiated bronchopulmonary carcinoma adn hypertrichosis lanuginosa as paraneoplasic expression of the tumor.
Since the 16th century there are well documented examples for cases of hypertrichosis universalis. They are often confused with legends and stories of wild men. The patients were often shown at courtyards and later on at fairs. The observation of a new case of hypertrichosis universalis lanuginosa combines the description of hairiness and teeth with its psychical and physical development. Our patient was socially intergrated ones kept from outsider position.
A case of hypertrichosis due to the hypotensive drug minoxidil is described. A review of the literature suggests that this complication appears in nearly all patients treated with this drug. The mechanism is unknown, but the similarity to the cases of hypertrichosis due to diazoxide, another potent vasodilator, suggests that increased cutaneous blood flow may be a factor.
After a recall of the etiologies of hypertrichosis and hirsutism, a method of diagnosis limited to functional pathological cases, was established. Having 31 complete case reports of functional hirsutism or hypertrichosis in women whose diagnosis--ovarian dystrophy or corticosuprarenal hyperplasia--was confirmed, the authors established a decision procedure based on the confrontation of two criteria obtained with a discriminant analysis program. These criteria included a clinical examination (intensity and localization of pilosity) and the effect of dexamethasone blockade on two plasmatic steroids, i. e., 17-hydroxyprogesterone and testosterone. The proposed diagnosis was furnished with a confidence index. 43 new cases confirmed the consistency between the clinical diagnosis and the automatic diagnosis.
A 54-year-old woman had acquired hypertrichosis lanuginosa. She developed excessive growth of lanugo-like hair all over her body two years prior to the sudden appearance of a rapidly growing, localized, solid mammary tumor. Of 12 published cases, nine have been associated with an internal malignant neoplasm and obviously, as in the present case, the cutaneous symptoms may serve as a skin marker by preceding the tumor diagnosis for up to two years.
We report on a boy with bilateral ectropion, ocular hypertelorism, bulbous nose, macrostomia with thin lips, abnormal ears, hypertrichosis of the forehead, neck and back, atrophic skin with hypoplastic nipples. Cause and inheritance are unknown.