1/9. 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) |
2/9. Repair of wide coup de sabre without cutaneous excision by means of pericranial-galeal padding flap.A case of double linear scleroderma of the forehead (coup de sabre) is described. The histopathology of this rare lesion is now well known with a normal epidermis and a sclerotic dermis. The correction was done with an original two-stage procedure: the lesion with alopecia was first treated by excision-suture and a transfer of the involved subcutaneous tissue along the right inner canthus; 1 year later, by a hemicoronal incision, we transferred a galeal-pericranial flap beneath the wider forehead lesion. We think that the use of a filling flap to correct wide coup de sabre lesions without cutaneous excision can be a simple alternative to the classic treatment by complete excision and flap reconstruction. The subcutaneous fascial system of the scalp can provide a good donor site with minimal morbidity.- - - - - - - - - - ranking = 1keywords = epidermis (Clic here for more details about this article) |
3/9. 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) |
4/9. High-frequency ultrasound as a useful device in the preliminary differentiation of lichen sclerosus et atrophicus from morphea.High-frequency ultrasound diagnostic equipment (HFUS) using probes with frequencies above 15-MHz has been utilized conventionally in preoperative assessments and postoperative follow-up of skin tumors. The advent of probes with even higher frequencies (up to 30-MHz) has widened the clinical applications of HFUS to inflammatory dermatoses which are histologically confined to the epidermis and upper dermis, such as psoriasis and atopic dermatitis. Ultrasound imaging has the advantage of being a noninvasive and relatively inexpensive technology that is quick and easy to perform. In addition, information obtained from HFUS is unique and useful for clinical diagnosis and evaluation for various skin disorders. We believe that HFUS has the potential to become a powerful tool for either clinical or investigative dermatology. Herein, we present a case of lichen sclerosus et atrophicus (LSA) to which we applied HFUS before skin biopsy and obtained substantial information for the differential diagnosis from morphea, and we reemphasize the usefulness of HFUS in studying the inflammatory skin disorders.- - - - - - - - - - ranking = 1keywords = epidermis (Clic here for more details about this article) |
5/9. A case of sclerodermatous graft-versus-host disease following autologous peripheral blood stem cell transplantation.We report a 65-year-old woman with chronic graft-versus-host disease (GVHD) who developed severely sclerotic skin on the fingers, hand and trunk following autologous peripheral blood stem cell transplantation (APBSCT). The patient had suffered from breast cancer and been treated with surgery and chemotherapy. She showed pancytopenia and was treated with APBCST. Four years after APBSCT, she developed the severe sclerotic changes on the fingers, hands, extremities and trunk. The skin biopsy showed a flattened epidermis and a proliferation of collagen bundles in the dermis. No anti-nucleolar dna titers were detected in the serum. She was diagnosed with chronic GVHD. Despite treatment with oral prednisolone, the skin sclerotic change developed and the breast cancer recurred. She died due to pericarditis. This is a rare case of sclerodermatous GVHD following APBSCT. The serum interleukin (IL)-12 levels were examined during the treatment.- - - - - - - - - - ranking = 1keywords = epidermis (Clic here for more details about this article) |
6/9. 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) |
7/9. Morphoea (localised scleroderma) in a patient with mixed connective tissue disease.An 18 year old girl concurrently developed skin lesions of morphoea (localised scleroderma) and pain and swelling of the hands and fingers. There were no dermatological or systemic signs of systemic sclerosis. The immunological features (high titred speckled antinuclear antibody, negative dna binding, high titred positive anti-RNP and negative anti-Sm antibodies, speckled nuclear Ig fluorescence in the epidermis of the skin lesions) were consistent with mixed connective tissue disease, and it is suggested that the morphoea represented a component of this condition.- - - - - - - - - - ranking = 1keywords = epidermis (Clic here for more details about this article) |
8/9. Morphea of the eyelids.Morphea, or localized scleroderma, of the eyelids is an uncommon disease. Morphea usually involves the thorax, trunk, lower and upper extremities, face, and genitalia. In the present report a patient with a biopsy-proven morphea of both upper eyelids is described. The salient histopathologic features included thinning of the epidermis with thickening and sclerosis of the collagen fibers in both the papillary and reticular dermis. There was a marked decrease in the fibrocytes. The eccrine sweat glands were entrapped by sclerotic collagen fibers. The pilosebaceous units were markedly decreased in number. There was a moderate lymphocytic infiltration in the dermis and a prominent lymphocytic perivasculitis. The clinical and histopathologic features of morphea are compared with those of lichen sclerosus et atrophicus.- - - - - - - - - - ranking = 1keywords = epidermis (Clic here for more details about this article) |
9/9. Lupus erythematosus panniculitis with morphea-like lesions.A 22-year-old female with morphea-like lesions, deep subcutaneous nodules and lipoatrophic areas of the skin on lateral aspects of the upper arms, on the breasts and on the buttocks is described. In 1990 a biopsy specimen obtained from a subcutaneous nodule showed hyaline necrosis of fat tissue; there were no epidermal changes. Direct immunofluorescence revealed granular deposits of IgM at the dermo-epidermal junction of the skin overlying the subcutaneous nodule. In a biopsy specimen taken at the onset of the disease in 1988, hyaline sclerosis of the deep dermis, follicular hyperkeratosis and vacuolar degeneration in the epidermis were described. There was weak positivity for antinuclear antibodies. The diagnosis of lupus erythematosus panniculitis (LEP) was made. Administration of chloroquine resulted in complete clearing of nodules in 3 months. The reported case demonstrates the difficulties in establishing the diagnosis of LEP in patients who present with subcutaneous disease, morphea-like lesions and who do not have other clinical or laboratory evidence of lupus erythematosus. The differential diagnosis of LEP and deep morphea is discussed.- - - - - - - - - - ranking = 1keywords = epidermis (Clic here for more details about this article) |