Cases reported "Dermatitis Herpetiformis"

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1/24. Generalized pustular psoriasis of pregnancy (impetigo herpetiformis).

    A 17-year-old woman had a sudden eruption of pustules in her intertriginous areas as well as of erythematosquamous plaques on the scalp, elbows, palms and soles in the third trimester of her first pregnancy. Histopathological evaluation of a biopsy revealed typical changes of pustular psoriasis with parakeratosis and abscesses of neutrophils (Kogoj's spongiform pustules). The diagnosis of pustular psoriasis was established by the typical clinical and histopathological findings. Laboratory parameters showed a highly elevated blood sedimentation rate, hypoferric anemia and decreased albumin levels. serum concentrations of parathormone and its metabolites were normal. After systemic treatment with glucocorticosteroids and antibiotics, the lesions improved but did not clear. After delivery of a healthy boy, therapy was switched to retinoid photochemotherapy with isotretinoin and PUVA that resulted in rapid and complete clearing of the eruption. The coincidence of plaque-type psoriasis and a pustular eruption as described previously in impetigo herpetiformis supports the view that this dermatosis of pregnancy is a variant of generalized pustular psoriasis.
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2/24. Palmar petechiae in dermatitis herpetiformis: a case report and clinical review.

    Palmar petechiae or purpura is an unusual finding in dermatitis herpetiformis (DH) that occurs in children but is only rarely reported in adults. We describe a 46-year-old man with DH who presented with the classic pruritic papulovesicular eruption and associated volar finger and palmar petechiae. We discuss recent advances in the pathogenesis and treatment of DH.
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3/24. A bullous neutrophilic dermatosis in a patient with severe rheumatoid arthritis and monoclonal IgA gammopathy.

    A 35-year-old man presented with a 2-month history of itchy grouped vesiculobullae with crusted papules and confluent plaques, distributed symmetrically on the thighs, knees, and elbows. The man had experienced severe disabling seropositive rheumatoid arthritis for 18 years previously. Histologically, subepidermal vesicles were noted, and a dense, diffuse neutrophilic infiltration of the dermis. Direct immunofluorescence studies did not detect IgA deposits. The patient responded well to dapsone (100 mg/d) but not to systemic steroids. This patient's eruption might have been an unusual manifestation of rheumatoid neutrophilic dermatitis.
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4/24. impetigo herpetiformis--a rare dermatosis of pregnancy associated with prenatal complications.

    INTRODUCTION: We describe a patient who developed an acute generalised pustular eruption associated with pyrexia at 33 weeks of gestation. CLINICAL PICTURE: Her condition was complicated by preterm labour, requiring an emergency caesarean section delivery. A diagnosis of impetigo herpetiformis was made on clinicopathological grounds. TREATMENT: Initial treatment with topical and systemic corticosteroids failed and oral methotrexate was added. OUTCOME: She responded well to this therapy and required only 2 doses of weekly methotrexate. CONCLUSION: impetigo herpetiformis is a rare dermatosis of pregnancy that requires early recognition and treatment to prevent maternal and fetal morbidity. Combination therapy using oral corticosteroids and short-term methotrexate is effective.
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5/24. Recurrent pericarditis and dermatitis herpetiformis. Evidence for immune complex deposition in the pericardium.

    Recurrent pericarditis can be associated with many chronic illnesses. Dermatitis herpetiformis is a chronic papulovesicular eruption which is characterized by granular IgA deposits in the dermal papillary tips and associated with a gluten-sensitive enteropathy. We describe the first case of recurrent pericarditis in association with dermatitis herpetiformis. This supposition is supported by exclusion of other possible etiologies and pericardial biopsy which revealed the deposition of IgG, IgA and complement.
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6/24. Immune complex disease and gynecomastia.

    A man with arthritis, gynecomastia and a rash histologically showing leukocytoclastic vasculitis, dermatitis herpetiformis and immunoglobulin g deposits at the dermoepidermal junction is reported. In contrast to cases of systemic lupus erythematosus (SLE) associated with vesiculobullous eruptions and similar histological features, our patient had neither bullae nor serological evidence of SLE. Conversely, no men already reported had evidence of feminization. Thus, our case appears to be unique.
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7/24. Papular dermatitis herpetiformis. Report of a case with localized, facial lesions.

    A male patient with unusual clinical features of dermatitis herpetiformis is reported. The eruption consisting mainly of erythematous papules developed symmetrically around the mouth and later around the eyes, and has been circumscribed within these areas for 4 years. The dermatitis herpetiformis nature of the lesion was confirmed by direct immunofluorescent testing.
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8/24. Unusual subepidermal bullous diseases with immunologic features of bullous pemphigoid.

    Sixty-seven patients with histologic and immunologic features of bullous pemphigold (BP) were evaluated. Eleven patients had a localized blistering disease that was predominantly confined to one area of the body, most commonly the lower extremities. Two patients displayed a dapsone-responsive blistering disease that was characterized by a flexural distribution of ten to 20 1-cm or less, intensely pruitic, subepidermal bullae and linear IgA basement membrane zone deposition. Two patients had a chronic recalcitrant generalized scarring, hyperkeratotic, subepidermal blistering eruption that demonstrated serologic and direct immunofluorescence (IF) findings of BP. One patient displayed grouped small vesicles surmounted on an erythematous base; the clinical diagnosis was dermatitis herpetiformis, but direct IF examination of the biopsy specimen showed features of BP. One patient with epidermolysis bullosa acquisita had serologic and direct IF features suggestive of BP.
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9/24. Polymorphic pemphigoid.

    We describe 20 patients with a chronic polymorphic eruption; they shared clinical, histopathological, and therapeutic features of both dermatitis herpetiformis and bullous pemphigoid (BP). In 14 of these 20 cases, direct and indirect immunofluorescence studies corresponded to BP. The remaining six patients showed IgA deposits in a linear pattern at the basement membrane zone, and two of these six showed IgA pemphigoid antibodies in their sera as well. No significant clinical and histological differences were detected in the patients, in connection with the immunological findings. Furthermore, one patient's condition, which was studied by repeated immunofluorescence examinations, changed from a linear IgA pattern and a negative indirect test to a linear IgG pattern and a positive reaction for IgG pemphigoid antibodies. We concluded that these cases constitute a polymorphic variant of BP.
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10/24. Herpetiform blisters in systemic lupus erythematosus.

    Four patients with systemic lupus erythematosus (SLE) had a vesicobullous eruption that histologically resembled dermatitis herpetiformis. Immunofluorescence microscopy patterns in the skin studied in three patients, however, were characteristic of SLE. Treatment of the underlying collagen-vascular disease improved the eruption, while recurrence coincided with exacerbation of the systemic disease. This distinctive lesion is an important parameter of SLE activity.
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