1/10. A unique variant of Darier's disease.A 45-year-old black woman presented with a chief complaint of an increasing number of "light spots" on her face, upper trunk, and legs. She had a 4-year history of a pruritic eruption on the dorsum of her hands. The eruption was particularly pruritic in the summer months. Other family members, including her sister and her daughters, reportedly had a similar dermatologic problem. The patient had been previously evaluated and biopsied by another dermatologist. The earlier biopsy was nondiagnostic, however, and she presented for further evaluation of this problem. On physical examination, the patient had hypopigmented macules along her jawline (Fig. 1), lateral neck, and upper chest. She had similar hypopigmented macules on her thighs. She had hyperkeratosis of the palmoplantar surface of her hands and feet. The dorsum of her hands had numerous coalescing, shiny, flat-topped, hypopigmented papules (Fig. 2), and several of her fingernails had distal, V-shaped notching. A punch biopsy from a papule on the dorsum of her hand was obtained. The epidermis had corps ronds present with focal areas of acantholysis above the basal layer (Fig. 3). The dermis had sparse, superficial, perivascular infiltrates composed of lymphocytes and histiocytes. These changes were consistent with our clinical diagnosis of Darier's disease (keratosis follicularis).- - - - - - - - - - ranking = 1keywords = epidermis (Clic here for more details about this article) |
2/10. Naevus anaemicus with teleangiectatic vessels.We describe a 20-year-old man with naevus anaemicus on the chest where, after dermabrasion of the epidermis, enlarged teleangiectatic dark-red vessels were seen within the previously pale area. They were clearly different from those seen on dermabrasion at this site in normal skin and in patients with vitiligo where the area is lighter red with only small punctual bleedings from arterial capillaries. The naevus anaemicus and a port-wine stain (naevus flammeus) in the same location is a phenomenon of vascular twin spotting, which was revealed when the epidermis was removed. The area was transplanted with thin epidermal grafts and healed within 2 weeks. One year later the naevus looked the same as before grafting. Much thicker grafts than those used by us will be needed, but they are not cosmetically acceptable.- - - - - - - - - - ranking = 2keywords = epidermis (Clic here for more details about this article) |
3/10. Hypermelanocytic guttate and macular segmental hypomelanosis.We report two sisters, 27 and 30 years of age, with a cutaneous pigmentary anomaly, which seems to be a new entity. At the age of 26 years the elder sister developed an asymptomatic and persistent rash consisting of discrete, grouped, round to oval, guttate and nummular, hypopigmented macules, 0.2-5 cm in diameter. The distribution of the lesions was unilateral. They were located on the right side of the thorax with a moderately sharp demarcation in the mid-line and ran in a segmental distribution over the right arm, hand and fingers. Microscopic examination of lesional skin scrapings was negative for fungi. Examination with wood's light accentuated the lesions from the surrounding normal skin. The younger sister had experienced identical, mostly guttate, skin lesions for many years, which at examination were distributed on all extremities and buttocks, and to a lesser degree on the trunk, but here in a segmental distribution. Histological examination (Masson-Fontana staining) of lesional skin of both sisters was identical. A slightly thinned epidermis and a marked decrease in pigmentation of the epidermal basal layer was seen. Electron microscopic examination of lesional skin showed an overall linear increase of morphologically and cytologically normal melanocytes just above the epidermal basal membrane. At many places the density of melanocytes was so high that the keratinocytes were displaced from the basal layer. The melanocytic dendrites extended into the suprabasal layer. The keratinocytes of lesional skin showed a decreased number of melanosomes. It is paradoxical that a hypomelanotic macule shows a histological picture of an increase in normal functioning melanocytes. In all probability a deficient melanosome transfer is responsible for this unexpected phenomenon.- - - - - - - - - - ranking = 1keywords = epidermis (Clic here for more details about this article) |
4/10. Selective decrease of eumelanin in hypopigmented epidermis of hypomelanosis of Ito.A Japanese infant had bilateral hypopigmented macules in a whorled or marble-cakelike configuration on her trunk and extremities. Her irides were bluish gray and her hair was dark brown. She had photophobia and macrocephaly with developmental delay. Analysis of peripheral lymphocytes and dermal fibroblasts disclosed a normal chromosomal pattern. A new finding was that the eumelanin content of the hypopigmented epidermis was decreased. We suggest that at least a portion of patients with this disorder have a defect in eumelanogenesis in hypopigmented skin.- - - - - - - - - - ranking = 5keywords = epidermis (Clic here for more details about this article) |
5/10. Brown papules and leukoderma in Darier's disease: clinical and histological features.We report a Japanese case of Darier's disease with brown and white papules or maculae distributed on the neck, trunk and dorsa of hands. Both brown papules and leukoderma showed typical histological features of Darier's disease. Moreover, there were much fewer melanocytes and melanosomes in the epidermis of both lesions. The corneal layer of the brown papules was far thicker than that of the leukoderma. Therefore, the thick corneal layer of the brown papules may prolong the retention of a few melanosomes or a little melanin to induce hyperpigmentation, while the thin corneal layer of leukoderma may not do so, thereby producing hypopigmentation. The difference in clinical and histological courses after involvement of melanocytes was proposed to be the cause of the discrepancy between the previous reports. We conclude that leukoderma was the primary lesion, the postinflammatory depigmented spots, or the atypical or subclinical eruption of Darier's disease.- - - - - - - - - - ranking = 1keywords = epidermis (Clic here for more details about this article) |
6/10. Histopathological evaluation of halo phenomenon in Spitz nevus.We saw a patient with a Spitz nevus surrounded by a halo of depigmentation. Although the halo phenomenon is often seen in pigmented melanocytic nevus, an association of this phenomenon with Spitz nevus seems to be rare. Moreover, histopathological findings showed focal lymphoid infiltration in the epidermis of the depigmented halo and marked infiltration in dermal epithelioid nevus cell nests. Thus, it is suggested that identical mechanisms are involved in the destruction of epidermal melanocytes and dermal nevus cells in halo Spitz nevus.- - - - - - - - - - ranking = 1keywords = epidermis (Clic here for more details about this article) |
7/10. Mammary Paget's disease confined to the areola and associated with multifocal Toker cell hyperplasia.A hitherto unreported variant of mammary Paget's disease (MPD) limited to the areola, leaving the nipple unaffected, has been analyzed by serial sectioning of the whole areola and nipple. This otherwise characteristic MPD proved to be confined to the epidermis. There was no underlying carcinoma. This MPD was associated with a multifocal presence of monomorphic but otherwise similar cells in small collections surrounding the ostia of areolar mammary glands in the clinically unaffected area. This condition was interpreted as hyperplasia of mammary gland-related cells also found in normal nipples (so-called Toker cells). The observations hint at a possible derivation of some cases of mammary and extramammary Paget's disease from such Toker cells.- - - - - - - - - - ranking = 1keywords = epidermis (Clic here for more details about this article) |
8/10. Analysis of the lymphocytic infiltrate in a case of vitiligo.We report a case of vitiligo notable for a degree and pattern of lymphocytic inflammation. hypopigmentation and subsequent depigmentation developed in a 65-year-old man. Initial skin biopsy specimen from the border of a patch of hypopigmentation revealed a moderately dense perivascular and interstitial infiltrate of lymphocytes with exocytosis into epidermis and follicular epithelium. The pattern of the infiltrate suggested the diagnosis of cutaneous T-cell lymphoma or connective-tissue disease. Immunophenotypic analysis revealed a mature T-cell population with retention of pan-T-cell markers. Analysis of the T-cell receptor genome did not identify a rearranged clone. Six months later, the clinical and histologic findings were typical in a noninflamed lesion of vitiligo.- - - - - - - - - - ranking = 1keywords = epidermis (Clic here for more details about this article) |
9/10. Clear cell papulosis of the skin.Clear cell papulosis is a new entity first described in 1987. To date, six patients have been reported: all were young Taiwanese children. The disease is characterized clinically by multiple small, whitish maculopapules distributed along the milk line and by the presence of large, benign pagetoid cells in the epidermis resembling the clear cell of the nipple. The significance of this entity lies in its potential histogenetic link with Paget's disease of the skin. We report four new Taiwanese patients, three girls and one boy, aged between 21 months and 4 years. Two were sisters. Small hypopigmented macules first appeared on the pubis. They were eventually distributed bilaterally along the milk line but were most numerous in the public area. The disease may easily be overlooked when the macules are tiny or few in number and thus display no clear milk-line distribution, or when they occur in white-skinned individuals. Histologically, solitary large clear cells with large, round pale nuclei were detected in the basal layer of the hypomelaninized epidermis. The numbers of clear cells varied on haematoxylin and eosin staining and were only small in two patients. The cytoplasm of the clear cells was decorated by antikeratin AE1 and anticarcinoembryonic antigen antibodies. AE1 was the best marker of the clear cell. Some of the AE1-positive cells were tadpole-like in shape and were situated well above the basal layer. Ultrastructurally, large clumps of disintegrated or vacuolated mucin granules were present in the cytoplasm of the clear cells. The melanocytes appeared normal; the suprabasal keratinocytes were essentially devoid of melanosomes. The pathological findings in the present study support the hypothesis that these clear cells are an aberrant derivative of sweat gland cells in the epidermis and are potentially the precursor cells giving rise to mammary and extramammary Paget's disease. The differential diagnosis includes chicken pox scars, idiopathic guttate hypomelanosis, hypomelanotic tinea versicolor, anetoderma and early, hypopigmented lesions of Paget's disease.- - - - - - - - - - ranking = 3keywords = epidermis (Clic here for more details about this article) |
10/10. child syndrome with linear eruptions, hypopigmented bands, and verruciform xanthoma.A Colombian girl was born with linear verrucous lesions of the right forearm and fingers, hypopigmented bands and streaks on the right arm, verrucous plaques on the right labium majus which extended to the right upper inner thigh, a crooked right tibia, nasal septum deviation to the right, lumbar kyphoscoliosis to the right, and other right-sided abnormalities. Radiographs showed stippled calcification of the tibial epiphysis, hypoplasia of the calcaneus, and astragalus of the right foot and delta phalanges of the right ring finger. Histologic examination demonstrated hyperkeratosis with ortho- and parakeratosis of the acanthotic epidermis. keratinocytes in the lower epidermis and connective tissue cells, schwann cells, and capillary endothelial cells of the papillary dermis showed marked vacuolization, a picture compatible with verruciform xanthoma. Electron microscopy revealed lipid vacuoles with lamellar contents and occasional crystals, compatible with cholesterol, in the dermal cells. Other abnormalities included a large number of vesicles and vacuoles in the upper epidermal keratinocytes and a lack of typical cementsomes; the vesicles and vacuoles were thought to represent abnormal cementsomes (lamellar granules).- - - - - - - - - - ranking = 2keywords = epidermis (Clic here for more details about this article) |
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