1/17. Hereditary perioral pigmented follicular atrophoderma associated with milia and epidermoid cysts.Eight members of a single family all presented the characteristic changes of facial, especially perioral, pigmented follicular atrophoderma, with numerous milia and epidermoid cysts. For this condition. diagnosis at a glance may be possible because of the perioral cutaneous manifestations. Histopathological examination of follicular atrophoderma revealed proliferation of basaloid cells continuous with the epidermis and coarse collagen fibres, with a decreased density of elastic fibres around the basaloid cells. Two of the eight individuals also showed generalized hypohidrosis. The eight affected persons were the proband, her son, mother, uncle, two younger sisters, cousin and nephew: an autosomal dominant mode of transmission was suggested from this family tree. The patients' symptoms resembled those of Bazex-Dupre-Christol syndrome, except for the different distribution of the follicular atrophoderma and the absence of basal cell carcinoma and hypotrichosis. This disease may be an entirely new syndrome characterized by perioral pigmented follicular atrophoderma associated with milia and epidermoid cysts.- - - - - - - - - - ranking = 1keywords = epidermis (Clic here for more details about this article) |
2/17. Pagetoid self-healing Langerhans cell histiocytosis in an infant.We report Langerhans cell (LC) histiocytosis in a male infant who developed numerous papular lesions on the trunk and posterior scalp soon after birth and spontaneously recovered from the disease within 7 months. Histologically S-100-positive cells were detected in the epidermis and papillary dermis, in some lesions mostly in the epidermis. Tumor cells in the epidermis were either clustered, forming nests, or scattered singly in pagetoid fashion. Electron microscopy confirmed the presence of Birbeck granules in these cells. They exhibited many interesting features usually not found in normal LCs, including mitosis, frequent apoptosis, Birbeck granules invaginated in the nucleus, autophagocytosis of Birbeck granules, and active ingestion of extracellular material through Birbeck granules attached to cell membranes. It is suggested that either a strong epidermotropism of tumor cells or a proliferation of the resident LCs of the epidermis is responsible for this intraepidermal growth pattern. Cellular necrosis through very active apoptosis and the superficial nature of the growth might have contributed to the self-healing course in this patient.- - - - - - - - - - ranking = 4keywords = epidermis (Clic here for more details about this article) |
3/17. A Japanese case of Kindler syndrome.A 25-year-old Japanese woman presented with contracture of the fingers and toes, and difficulty in opening her mouth. Her grandparents are first cousins, but none of the other members of the family are affected. Bulla formation started at birth on areas of the skin that received pressure, and in infancy and early childhood the lesions were limited only to the acral areas. She also had bilateral, incomplete syndactylies involving all web spaces (Fig. 1a). The formation of blisters ceased after the age of 15 years, but a generalized progressive poikiloderma then appeared with accompanying cutaneous atrophy of the skin of the neck, trunk, and extremities (Fig. 1b). The patient experienced mild photosensitivity of the face and neck. At age 18 years, surgical removal of the webbing of all her fingers was performed. Oral examination showed atrophy of the buccal mucosa, and an inability to fully open the mouth. The patient also suffered from poor dentition and easily bleeding gums, but had no symptoms of esophageal dysfunction. histology of separate biopsy specimens, taken from the poikilodermatous pretibial and trunk skin, showed classical features of poikiloderma, namely epidermal atrophy with flattening of the rete ridges, vacuolization of basal keratinocytes, pigmentary incontinence, and mild dermal perivascularization (Fig. 2a). Interestingly, dyskeratotic cells (Fig. 2b) and eosinophilic rounded bodies (colloid bodies) (Fig. 2c) were frequently found at the basal keratinocyte layer and in the upper dermis, respectively. Pigment was also present in the upper epidermis. To rule out the possibility of a congenital epidermolysis bullosa, ultrastructural and immunofluorescence studies were performed. Ultrastructural studies demonstrated the reduplication of the basal lamina with branching structures within the upper dermis and cleavage between the lamina densa and the cell membrane of the keratinocytes (Fig. 3a). The numbers of associated anchoring fibrils did not seem to be reduced, and colloid bodies and dyskeratotic cells were detected. Immunofluorescence studies with the antibody against type VII collagen (LH 7 : 2) were subsequently carried out. The results showed extensive broad bands with intermittently discontinuous and reticular staining at the dermo-epidermal junction (DEJ) (Fig. 3b), whereas a linear distribution is typically seen in healthy tissue (data not shown). Interestingly, direct immunofluorescence studies revealed intracellular accumulation of immunoglobulin g (IgG), IgM, IgA, and C3 in colloid bodies under the basement membrane (Fig. 3c).- - - - - - - - - - ranking = 1keywords = epidermis (Clic here for more details about this article) |
4/17. Mandibular melasma.Melasma is an acquired symmetrical brown hypermelanosis of the face. Three clinical patterns are recognized, namely centrofacial, malar and mandibular. This study addresses the clinical and histopathological features of ten women with mandibular melasma to determine its relation to other types of melasma. The mean age of the patients was 52 years and the onset of the disease occurred at an average age of 44 years. sunlight aggravated the pigmentation in all cases. Only one patient was receiving hormonal therapy. Histopathologically, all patients demonstrated severe sundamage and hyperpigmentation of the epidermis. Nine of them had melanophages or melanin in the papillary dermis. We conclude than mandibular melasma may be a clinicopathologic entity different to other types of melasma.- - - - - - - - - - ranking = 1keywords = epidermis (Clic here for more details about this article) |
5/17. Atrophoderma of moulin with preceding inflammation.A 16-year-old Vietnamese man presented to the dermatology Clinic with a 10-year history of bizarre brown patches, which initially started as red asymptomatic "bumps" on the trunk, upper and lower extremities, and face. His past medical history was significant for hypothyroidism and idiopathic urticaria. He was on Eltroxin for hypothyroidism. The family history was noncontributory. physical examination revealed two types of lesion: erythematous, well-circumscribed papules in a linear configuration along with linear hyperpigmented atrophic patches following Blaschko's lines were noted on the lower extremities (Fig. 1), right upper extremity, right flank (Fig. 2), and right jawline. Initial biopsies taken from the papular lesions on the right thigh and right elbow revealed the following changes. The first biopsy showed a slightly thinned epidermis with prominent dilated blood vessels in the superficial dermis. There also appeared to be a slight increase in the amount of collagen in the deep dermis. The findings were reported as in keeping with "epithelial atrophy." The second biopsy from the lesion on the right elbow revealed an acanthotic epidermis. The granular layer was absent in several areas and there was marked overlying parakeratosis. In the dermis, there was a heavy perivascular lymphocytic infiltrate. The appearances were consistent with a psoriasiform dermatitis (Fig. 3). A biopsy taken from the left thigh approximately 18 months later showed slight irregular acanthosis with dermal edema, dilated blood vessels, and a patchy lymphocytic infiltrate. The appearances were compatible with mild inflammation.- - - - - - - - - - ranking = 2keywords = epidermis (Clic here for more details about this article) |
6/17. Linear atrophoderma of Moulin.We report a typical case of linear atrophoderma of Moulin that represents a distinct clinical entity. A 17-year-old woman presented with hyperpigmented and atrophic band-like skin lesions measuring 3-5 cm in breadth on the right side of her trunk and on the right buttock, in an arrangement following the system of Blaschko's lines. The skin lesions had a normal texture and showed no signs of inflammation, lilac ring, erythema, induration, sclerosis or depigmentation. Routine laboratory data were normal. Antinuclear antibodies and anti-Scl70-antibodies were negative. Histopathologically, a moderate diffuse hyperpigmentation within the lower epidermis, a focal vacuolar degeneration of the basal layer, and a few dermal perivascular lymphocytes accompanied by signs of pigment incontinence were noted. Intravenous penicillin g was administered for 14 days in a dosage of 10 x 10(6) IU twice daily. This treatment was repeated after 3 and 9 months. No effect was noted. Linear atrophoderma of Moulin takes a chronic course without progression or regression. The disease leads to significant cosmetic impairment and this may cause emotional stress. Due to its relationship to idiopathic atrophoderma of Pierini-Pasini and linear scleroderma, treatment with intravenous penicillin was tried. In our case it had no effect.- - - - - - - - - - ranking = 1keywords = epidermis (Clic here for more details about this article) |
7/17. En coup de sabre.A man born in 1961 was apparently well until his marriage in May 1986. In August of that year, his attention was drawn to the presence of a reddish patch over the right forehead. It was initially asymptomatic. It continued to progress to form an apparent linear furrow extending from the receding hairline to the vertex and temporal part of the scalp. At this juncture, the patient experienced moderate pain when laughing. Five years later the patient experienced a constant boring pain affecting the right eyeball. Consultation with an ophthalmologist led to sacrificial enucleation of the right eye despite the lack of a precise diagnosis. Nevertheless, the initial condition continued to progress causing disfigurement of the right side of the face. Examination of the face was marked by perceptible asymmetry. A linear atrophic plaque in the form of a furrow was identified on the right side of the forehead extending from the eyebrow to the vertex and temporal part of the scalp. The skin over the furrow was taut and bound down (Fig. 1). A hematoxylin and eosin-stained section prepared from the lesion revealed marked thickening of the dermis. The collagen bundles were hypertrophied and closely packed together. The staining was homogeneously eosinophilic. It was largely devoid of inflammatory infiltrate. The changes in the blood vessels were conspicuous by narrowing of the lumina, thickening of the walls, and a sparse perivascular lymphocytic infiltrate. Pilosebaceous units were completely absent, whereas a few atrophic pulled-up sweat glands were located in the mid-dermis. Similar changes were observed in the subcutaneous tissue. The epidermis was largely atrophied, with flattening of the rete ridges.- - - - - - - - - - ranking = 1keywords = epidermis (Clic here for more details about this article) |
8/17. Idiopathic atrophie blanche.A 41-year-old woman presented with a 3-year history of purpuric lesions followed by superficial, painful ulcers and development of lesions on the lower legs and on the dorsa of the feet, particularly in the summer. The patient was asymptomatic during the winter months. On physical examination she had irregular, scleroatrophic, white-ivory, coalescent lesions on a livedoid basis, with purpuric and, in some lesions, pigmented borders with numerous telangiectatic capillaries. These lesions were localized on the medial sides of the lower legs and on the dorsa of the feet (Figure 1). Laboratory investigations were normal or negative, including complete blood cell count, platelets, coagulation indexes, erythrocyte sedimentation rate, serum immunoglobulins, antinuclear antibodies, anti-double-stranded dna, anticardiolipin, antiphospholipids, antineutrophilic cytoplasmic antibodies, circulating immunocomplexes, complement fractions (C3, C4), cryoglobulins, rheumatoid factor, and Rose-Waaler reaction. The only laboratory abnormality was an elevated fibrinogen level (472 mg/dL). Doppler velocimetry excluded a chronic venous insufficiency. Thoracic x-ray and abdominal ultrasound were normal. A digital photoplethysmograph revealed functional Raynaud's phenomenon. A biopsy specimen taken from a purpuric lesion showed an atrophic epidermis with parakeratosis and focal spongiosis. An increased number of small-sized vessels were observed within a sclerotic dermis. Most of the vessels in the upper dermis were dilated and showed endothelial swelling; some were occluded due to amorphous hyaline microthrombi (Figure 2). There were fibrinoid deposits around the vessels with thickening of the vessel walls. Extravasated erythrocytes were found throughout the upper and mid-dermis. There was a sparse perivascular lymphocytic infiltrate but no vasculitis. Direct immunofluorescence showed a perivascular microgranular deposit of IgM ( ), C3 ( ), and fibrinogen/fibrin ( ). On the basis of clinical, serologic, histopathologic, and immunopathologic findings, a diagnosis of idiopathic atrophie blanche was made. The patient was treated with dapsone (50 mg p.o. q.d.) and pentoxifylline (400 mg p.o. t.i.d.) with pain relief and complete resolution of the ulcerations after 6 weeks of therapy.- - - - - - - - - - ranking = 1keywords = epidermis (Clic here for more details about this article) |
9/17. lichen nitidus: a histologic and electron microscopic study.A lesion of lichen nitidus in a 53-year-old man was examined by histology and electron microscopy. The histology showed typical features. The electron microscopy revealed primary changes in hydropic degeneration of basal cells which possibly indicated severe edema between the basal keratinocytes and in the subepidermal area. The basal keratinocytes themselves were not particularly altered, except for clustered micro-vacuolar structures in a small portion of the peripheral cytoplasm. In one of the eight blocks observed, the cytoplasmic process of a mast cell was found between the basal keratinocytes, indicating that mast cells may possibly infiltrate the epidermis in lichen nitidus. Scattered mast cells in the dermis were also noted. Thus primary pathologic changes of lichen nitidus may be induced by severe edema, which was accompanied by inflammatory cells including mast cells, in the region of the dermal-epidermal junction.- - - - - - - - - - ranking = 1keywords = epidermis (Clic here for more details about this article) |
10/17. Atrophoderma (Pasini-Pierini). Findings on direct immunofluorescent, monoclonal antibody, and ultrastructural studies.A patient with atrophoderma (Pasini-Pierini) was studied. Microscopic examination showed small collections of mononuclear cells around dermal blood vessels. Electron microscopic study demonstrated macrophages and lymphocytes around vessels and between fibers in the dermis; the epidermis, dermis, collagen, and elastic fibers appeared normal. Monoclonal antibody studies of the cells in the perivascular infiltrate demonstrated cells reacting with anti-Leu-1 (pan-T-cell antibody), anti-Leu-3a (the helper/inducer T-cell antibody), and OKM 1 antibody-reacting cells (macrophages). Direct immunofluorescent studies showed IgM and C3 staining in the small blood vessels of the papillary dermis, scattered IgM cytoids at the basement membrane, and focal fibrinogen in the mid-dermis. Mononuclear cells in the perivascular infiltrate, similar in type and percentage concentration, have been demonstrated also in patients with anetoderma, another rare atrophic cutaneous disorder. macrophages and T lymphocytes around papillary dermal blood vessels may play a role in the pathogenesis of atrophoderma and anetoderma.- - - - - - - - - - ranking = 1keywords = epidermis (Clic here for more details about this article) |
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