Cases reported "Facial Dermatoses"

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1/70. 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.
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keywords = dermis
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2/70. granuloma faciale with extrafacial lesions.

    Extrafacial involvement in granuloma faciale (GF) is rather exceptional. We report herein a patient with GF associated with lesions on the trunk and the forearm. Histological studies of facial and extrafacial lesions shared similar characteristics: a mixed inflammatory infiltrate with abundant eosinophils in the superficial and middle dermis with a narrow grenz zone of uninvolved dermis between the epidermis and the infiltrate. Evidence of vasculitis was clearly observed in both biopsy specimens. Treatment with dapsone did not alter the course of the disease. We review the 12 cases of extrafacial GF that have been reported in the English and Spanish literature. In these cases a diagnosis of erythema elevatum diutinum (EED) may be suggested. Although GF and EED may share some pathogenic mechanisms, there are several clinical and histological differences between them that make us consider EED and GF as distinct entities.
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keywords = dermis
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3/70. The hyperkeratotic variant of disseminated superficial actinic porokeratosis (DSAP).

    A 78-year-old South Korean man was referred to us from the Medical intensive care Unit (MICU) for an opinion. He was comatose and was on ventilatory care due to aspiration pneumonia. Multiple tiny papules had developed 10 years previously and since then the number and size had been increasing gradually. He had been diabetic for the past 4 years, and had Parkinson's disease diagnosed 1 year previously. Laboratory examinations revealed an elevated level of white blood cells (WBCs) (25,000/microL) and decreased hemoglobin (8.8 g/dL). Other laboratory results were negative or within normal limits. skin examination showed multiple, discrete, crust-like, brownish papules over the erythematous base on the face, upper extremities, and lower extremities. With the clinical impressions of irritated verruca vulgaris, seborrheic keratosis, or cutaneous fungal infection, a skin biopsy was taken from a papule on the left shin, and histopathologic examination revealed several pronounced hyperkeratotic and parakeratotic columns, and characteristic cornoid lamellae in the stratum corneum. Beneath the cornoid lamellae, the granular layer was decreased. A number of round or oval, dyskeratotic, homogenized eosinophilic cells with pyknotic nuclei were scattered in the prickle cell layer below the cornoid lamellae. A mild lymphohistiocytic infiltrate was observed in the papillary dermis and around the blood vessels in the upper dermis. Also, actinic degeneration was present in the upper dermis.
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keywords = dermis
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4/70. The relationship between facial annular erythema and anti-SS-A/Ro antibodies in three East Asian women.

    A distinct annular erythema developed on the cheeks of three East Asian women who had anti-SS-A/Ro (SSA) antibodies. The erythema was characterized by a wide, elevated border and central pallor. Histologically, there was a coat-sleeve-like infiltration of lymphocytes around the blood vessels, appendages, and secretory gland cells in the dermis. Immunohistological analysis clarified that the majority of infiltrating lymphocytes were CD4-positive T cells. Abnormal expression of hla-dr antigens in the perivascular, appendage, and secretory gland cells in the dermis was also observed. The differential diagnosis of the three patients lay between Sjogren syndrome (SjS), Sjogren/systemic lupus erythematosus overlap syndrome and an asymptomatic clinical state. These results are consistent with recent findings of major histocompatibility complex class II expression on target organs in various autoimmune diseases. Based on these findings, erythema appears to represent a broad cutaneous manifestation of these diseases. Furthermore, the presence of SSA antibodies, aberrant HLA-DR expression, and sun exposure may be responsible for the development of erythema.
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keywords = dermis
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5/70. Lichenoid tissue reaction in porphyria cutanea tarda.

    We report a patient presenting with lichenoid plaques on exposed skin who had the metabolic features of porphyria cutanea tarda (PCT). histology of lesional skin demonstrated a lichenoid inflammatory cell infiltrate in the upper dermis, while direct immunofluorescence revealed immunoreactive colloid bodies. Monochromator irradiation testing demonstrated photosensitivity in the visible spectrum consistent with porphyria. Solar-simulated irradiation induced a papular reaction with lichenoid histological changes. We propose that this atypical presentation of PCT may reflect a lichenoid tissue response to a porphyrin-mediated photochemical reaction.
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keywords = dermis
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6/70. Sinus histiocytosis (Rosai-Dorfman disease) clinically limited to the skin.

    Sinus histiocytosis with massive lymphadenopathy (SHML) is an idiopathic proliferation of unique histiocytes that have vesicular nuclei and voluminous pale cytoplasm, often with emperipolesis. Pure cutaneous involvement is very rare. We describe a patient with SHML limited to the skin whose lesion has spontaneously regressed. A 35-year-old Korean male visited the Department of dermatology due to facial rash for 2 months. A 3 x 3.5 cm-sized well-demarcated dark erythematous nontender plaque was noted on the right cheek. skin biopsy showed dense, nodular infiltrates of histiocytes with abundant cytoplasm and vesicular nuclei rimmed by lymphoplasma cell aggregates throughout the upper and mid-dermis. The histiocytes were immunohistochemically positive for S-100 protein and CD68, but negative for CD1a. Laboratory tests and a thorough physical examination revealed no abnormalities. These findings suggested that this was a case of SHML clinically limited to the skin. The skin lesion was initially resistant to steroid therapy, but began to regress 10 months after the onset without further treatment.
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7/70. pemphigus foliaceus successfully treated with mycophenolate mofetil as a steroid-sparing agent.

    pemphigus foliaceus is an autoimmune blistering disease of unknown origin with antibodies produced against desmoglein 1, an adhesive protein found in the desmosomal cell junction in the suprabasal layers of the epidermis. The disease is primarily treated with corticosteroids and corticosteroid-sparing immunosuppressive agents. We report a case of pemphigus foliaceus successfully treated with mycophenolate mofetil. It remains to be seen whether this agent has a significant effect on the course of the disease and remission induction.
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keywords = dermis
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8/70. Solid facial edema in a patient with rosacea.

    We report a 53-year old man with symmetrical nonpitting edema, conjunctivitis, and acneiform eruptions on the face. Histopathological examination showed perifollicular lymphohistiocytic infiltration and telangiectasias in the upper dermis. Loosely aggregated non-caseating granulomas were scattered through the dermis; some of them were seen in the perifollicular regions. The patient was treated with fleroxacin (100 mg/day, orally) for two weeks with a marked reduction of both solid facial edema and periorbital edema.
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ranking = 2
keywords = dermis
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9/70. Discoid lupus erythematosus (DLE)-like lesion induced by uracil-tegafur (UFT).

    A mixture of uracil and tegafur (UFT) is a common antineoplastic agent in japan. We report a 64-year-old Japanese woman with discoid lupus erythematosus (DLE)-like lesions which were induced by UFT. After surgery to treat lung cancer, UFT (300 mg/day) was administered and she developed round erythema on her right cheek. A skin biopsy specimen taken from the right cheek site revealed atrophy of the epidermis, a slight liquefaction of the basal cell layer, and patchy lymphocytic infiltration in the perivascular and perifollicular regions. A test for antinuclear antibody was weakly positive (80 fold), and rheumatoid factor was slightly elevated (7.6 IU/ml). After discontinuation of UFT, the erythema completely regressed within 2 months. We reviewed 17 cases of DLE-like lesions induced by fluorouracil agents and summarized the common features.
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keywords = dermis
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10/70. Idiopathic eruptive macular pigmentation: report of 10 cases.

    Idiopathic eruptive macular pigmentation (IEMP) is a rare disease. IEMP is characterized by asymptomatic, pigmented macules involving the neck, trunk, and proximal extremities. This study describes 10 cases of idiopathic eruptive macular pigmentation seen during a 9-year period at the Asan Medical Center, Seoul, korea. We present these characteristic consecutive cases to provide more insight into the clinical picture and course of IEMP. skin lesions of 8 patients were multiple brown macules involving the trunk, face, neck, and extremities. In 2 patients, multiple dark brown macules and patches were noted. The age of onset varied from 1 to 20 years. Tentative diagnoses were usually ashy dermatosis (erythema dyschromicum perstans), fixed drug eruption, or mastocytosis. The history of any erythema and drug medication was absent. Darier's sign was absent. skin biopsy specimens showed increased pigmentation of the basal layer in an otherwise normal epidermis. Pigmentary incontinence, melanophages, and mild perivascular lymphohistiocytic infiltrate in the papillary dermis were also revealed. mast cells could not be found. The lesions gradually disappeared during a period of several months to years. The alleged rarity of IEMP may be partially caused by medical unfamiliarity with this entity, despite its clinical and histopathologic characteristic picture. Treatment of IEMP is unnecessary because spontaneous resolution of the lesions can be expected within several months to a few years.
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keywords = dermis
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