Upper dermal elastolysis: a comparative study with mid-dermal elastolysis.

We encountered a patient who complained of many small papules on the neck, shoulders, upper chest and upper back. Biopsy specimens showed complete loss of elastic fibers in the upper dermis including papillary dermis, whereas those of the mid dermis were intact. Electron microscopy revealed that assembly of component fibrils of elastic fibers was loose, and electron dense substance was aggregated in the spaces between these loosely bound subunit fibrils or along the periphery of abnormal fibers. Dermal phagocytes engulfed abnormal as well as normal elastic fibers. Upper dermal elastolysis is a clinical and histopathological entity different from mid-dermal elastolysis. Ultrastructural changes of the former are essentially similar to those of the latter but much more severe. It is suggested that activated elastophagocytosis of dermal phagocytes may play a role in this disease.

Imipramine hyperpigmentation: a slate-gray discoloration caused by long-term imipramine administration.

A 48-year-old white woman, skin type III, had a slate-gray discoloration of the face and dorsa of both hands after ingesting imipramine, 150 mg/day for 5 years. Her iris color was also darkened. One year after cessation of the therapy, the discoloration became lighter. Sun-protected skin showed no discoloration. Light microscopy revealed an accumulation of doubly refractile golden yellow granules in the papillary dermis, mostly scattered, with some concentration around the blood vessels but not in the endothelial cells. Electron micrographs showed numerous amorphous electron-dense inclusion bodies in histiocytes, phagocytes, fibroblasts, and dermal dendrocytes. Melanosomes were phagocytosed in the same cells but in separated locations. Imipramine is structurally related to chlorpromazine and can cause slate-gray discoloration. However, the color of the granules deposited in the papillary dermis is golden-yellow and they are not deposited in endothelial cells.

Localization of eosinophil granule major basic protein in incontinentia pigmenti.

We report a case of incontinentia pigmenti and demonstrate the deposition and localization of eosinophil major basic protein (MBP) in the vesicular stage of this neurocutaneous syndrome. The initial stage of incontinentia pigmenti is histologically characterized by intraepidermal vesicles associated with eosinophilic spongiotic dermatitis. Pathologic examination of a lesional tissue specimen from our patient demonstrated epidermal necrosis, spongiosis, and vesicle formation. Indirect immunofluorescence with affinity-chromatography purified antibody to human eosinophil granule MBP demonstrated many intact eosinophils within vesicles and scattered throughout the epidermis and dermis, and extracellular deposition of granular MBP in the tissue. The characteristic finding of extensive tissue eosinophilia in incontinentia pigmenti, as well as the new finding of extracellular deposition of an eosinophil granule protein in lesional tissue, suggests the involvement of eosinophils in the pathogenesis of the disease.

Partial genetic deficiency of the C4 component of complement in discoid lupus erythematosus and urticaria/angioedema.

A high incidence of discoid lupus erythematosus (DLE) and urticaria/angioedema was found among patients with selective low levels of the C4 component of complement. Although evidence for activation of C4 was present in patients' sera, studies to determine the presence of known activation mechanisms were negative. Genetic C4 typing in four pedigrees showed that all propositi carried the null allele B*QO. It is postulated that the low C4 levels in these patients are related to the skin lesions and this partial genetic deficiency.

Selective depression of the C4 component of complement: evidence for an association with genetic deficiency of C4 and dermatologic diseases.

Thirty-four patients with selective low serum C4 levels were found among 1,731 patients studied in a hospital-based routine clinical immunology laboratory. A high incidence of dermatologic diseases, particularly discoid lupus erythematosus and angioedema/urticaria, was present among patients with selective low C4. Although evidence for activation of C4 was present in patients' sera, studies to determine the presence of known activation mechanisms of complement were negative. Genetic C4 typing in four pedigrees showed that all propositi carried the null allele B*QO. It is postulated that the selective C4 depression in these patients is related to the skin lesions and this partial genetic deficiency.