E. Dorinda Shelley

Uncombable hair syndrome: Observations on response to biotin and occurrence in siblings with ectodermal dysplasia

Three children are reported with uncombable hair syndrome, consisting of slow-growing, straw-colored scalp hair that could not be combed flat. The hairs appeared normal on light microscopy but on scanning electron microscopy were triangular in cross section, with canal-like longitudinal depressions. Oral biotin, 0.3 mg three times a day, produced significant improvement after 4 months in one patient, with increased growth rate and with strength and combability of the hair, although the triangular shape remained. The other two patients were unique in having associated ectodermal dysplasia. Their hair slowly improved in appearance and combability over 5 years without biotin therapy.

Argyria: the intradermal "photograph," a manifestation of passive photosensitivity.

X-ray microprobe and electron microscopic study was made of the remarkable blue-black pigmentation that sunlight elicits in patients with argyria. The patient under study had developed argyria following injection of silver nitrate as a sclerosant into his varicose veins 41 years ago. Similarities are demonstrated between the darkening of the skin and the darkening of a photographic film following light exposure. In both instances, colorless silver salts and compounds present in an inert matrix (collagen versus gelatin) are reduced by incident light to black metallic silver. This passive photosensitivity reaction leads to silver tattooing of the light-exposed skin and to photographic imaging in the film.

Painful Fingers, Heat Intolerance, and Telangiectases of the Ear: Easily Ignored Childhood Signs of Fabry Disease

Abstract: Generalized anhldrosls with heat collapse, painful fingers, and angiokeratomas were the presenting signs in a 23‐year‐old man who was shown by enzyme studies and electron microscopy to have Fabry disease. His 6‐year‐old brother had the early diagnostic findings of episodic painful fingers, heat intolerance, and retroaurlcular telangiectasia. Both had an absence of α‐galactosidase, Over 6 years of observation, the older brother developed progressive renal failure and the younger one developed acrodynla and anhidrosls. Their mother had dlminished α‐galactosidase activity and several anglomatous papules on one breast A review of Fabry disease emphasizes the need for skin Inspection for anglomas and telanglectasia, and enzyme assay in patlents with lnexplicable complaints or lindings. Carrier females are most easily recognized by the presence of unique comeal opacities.

Long-term antibiotic therapy for balanitis xerotica obliterans

In an open uncontrolled study of 3 patients with balanitis xerotica obliterans we have observed significant improvement after long-term systemic antibiotic therapy. Two of the patients noticed softening of the skin as well as disappearance of pruritus, tenderness, and inflammatory changes within 3 weeks of receiving oral and intramuscular penicillin. The third patient experienced the same degree of improvement after a regimen of dirithromycin, 500 mg/day. Stopping the antiobiotic for 1 month resulted in relapse, with improvement again on resumption.

Aquadynia: Noradrenergic pain induced by bathing and responsive to clonidine

We describe two women in whom bathing was regularly followed by intense, widespread burning pain that lasted 15 to 45 minutes, which we term aquadynia. This water-induced pain was not caused by any skin or systemic diseases. We view it as a type of noradrenergic pain induced by contact with water, because it can be blocked with clonidine or propranolol. It appears to be an extension of the phenomenon of aquagenic pruritus, which we have also found to respond to clonidine or propranolol.

Essential progressive telangiectasia in an autoimmune setting: Successful treatment with acyclovir

A unique, progressive syndrome of muscle weakness, ascending telangiectasia, and venous ectasia of the legs developed in a 55-year-old woman with autoimmune thyroiditis. Myopathy was documented by electromyography and electron microscopy. Her constellation of findings is considered to be of autoimmune origin. Treatment with acyclovir eliminated the patients generalized telangiectasia. Cyclosporine therapy resulted in a marked but temporary reduction of the enlarged thyroid and of her elevated thyroglobulin and microsomal antibodies. Neither acyclovir nor cyclosporine had any demonstrable effect on the patients muscle function or venous ectasia.

Cutaneous Histiocytosis with Langerhans Cell eatures Induced by Scabies: A Case Report

Abstract: An infant with biopsy‐proven scabies developed nodular lesions. Histopathology revealed atypical histiocytes with Langerhans cell features. Within six months after treatment all skin lesions gradually disappeared. We suggest that the nodules In scabies can be due to Langerhans cell proliferation.

The infected hairs of tinea capitis due to Microsporum canis: Demonstration of uniqueness of the hair cuticle by scanning electron microscopy

Scanning and transmission electron microscopic studies were done on scalp hairs of four patients infected with ectothrix Microsporum canis. Both freeze fracture and cross-sectioning of hairs revealed a thick sheath of fungal spores encircling the hair shaft beneath an intact cuticle. These spores were not visible on surface inspection but became apparent only where the cuticle had been rubbed off or broken. Daily selenium sulfide shampoos removed all of the spores from these sites. The cuticle is viewed as being an effective barrier to the penetration of fungi, so that the hair is vulnerable to fungus infection only deep within the hair follicle below the level of the mature cuticle. Once the fungus enters the hair cortex just above the hair bulb, it produces myriads of spores that remain trapped and hidden beneath the cuticle for the length of the intact hair.

Immunization, Abrasion, and Sunburn as Localizing Factors in Chicken Pox

Abstract: Three children had striking localization of varicella lesions to skin overlying a diphtheria‐pertussis‐tetanus toxoid (DPT) immunization injection site, a facial abrasion, and a sunburn of the trunk, respectively, Presumably, during the viremic stage of varicella, the virus passed from damaged blood vessels into the skin. A disabling myalgia in the thigh at the DPT site in one child may also have been the result of localization of the varicella virus in the muscle during the inapparent viremic stage. This suggests that an injection given during an occult viremia could trigger an unexpected reaction al the site.

Blisters of the fingertips: A variant of bullous dermatosis of hemodialysis

Recurrent, deep, painful, noninflammatory blisters on the volar fingertips proved to be a variant of bullous dermatosis of hemodialysis.