| 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. ( info) |
2/148. pemphigus vulgaris preceded by herpetiform-like skin lesions with negative immunofluorescence findings. We report a case of pemphigus vulgaris in a 63-year-old woman. At the first onset, she developed herpetiform-like skin lesions. Their histology showed eosinophilic spongiosis and intraepidermal blister formation with an infiltrate of eosinophils. Both direct and indirect immunofluorescence studies were negative. She remained in remission for 6 years. At the second onset, she developed bullous skin lesions; histological examination disclosed suprabasal acantholysis and infiltration of eosinophils. Direct immunofluorescence revealed IgG and C3 deposits at the cell surface, predominantly in the lower epidermis. Indirect immunofluorescence using normal human skin as a substrate demonstrated anti-cell surface IgG antibodies at a titer of 1:160. Immunoblot analysis showed that the patient's serum reacted only with a 130 kD protein. This case indicates that pemphigus vulgaris can follow herpetiform-like skin lesions with negative immunofluorescence findings after a long remission time. ( info) |
3/148. impetigo herpetiformis during the puerperium. We report on a 29-year-old primigravida who developed impetigo herpetiformis 1 day after delivery. To our knowledge, this patient is the second reported case of impetigo herpetiformis presenting during the puerperium. The patient responded quickly to systemic administration of methotrexate and prednisolone. ( info) |
| Childhood dermatitis herpetiformis (DH) is rare. The true prevalence and incidence of this condition are unknown. We report a 7-year-old boy presenting with nonpruritic inflammatory papules on the buttocks and extensor surfaces, clinico-pathologically consistent with Sweet's neutrophilic dermatosis. Immunofluorescence studies showed IgA deposits in the dermal papillae consistent with DH. Remission was achieved with a gluten-free diet and dapsone. Childhood DH may present different clinical signs to the adult form and misdiagnosis can occur if immunofluorescence is not requested on skin biopsy. ( info) |
| A patient had a blistering and severely itching disease confined solely to the oral cavity. The histopathologic findings had the features of both pemphigus and dermatitis herpetiformis. There were no gastrointestinal symptoms, no IgA could be found in uninvolved skin, and no intercellular or basement membrane antibodies were present in the serum or epidermis. The disease responded favorable to dapsone (diaminodiphenylsulfone), which could be discontinued after 2 1/2 years. The literature is briefly reviewed, and there is a discussion of whether the patient was suffering from pemphigus or dermatitis herpetiformis. The conclusion is drawn that the diagnosis cannot be established with certainty and that we are perhaps dealing with a new disease. It is stressed that in such aberrant cases a therapeutic trial with dapsone should be made. ( info) |
| We report a patient with severe dermatitis herpetiformis (DH) who was intolerant of dapsone, sulphapyridine, systemic steroids, and azathioprine. He was treated effectively with a combination of heparin, tetracycline and nicotinamide. ( info) |
7/148. A case of dermatitis herpetiformis with IgA endomysial antibodies but negative direct immunofluorescent findings. A patient with clinical findings of dermatitis herpetiformis (DH), negative direct immunofluorescent (DIF) findings for junctional IgA deposits in 2 biopsy specimens, and positive for IgA endomysial (AEmA) and tissue transglutaminase (tTG) antibodies responded initially to dapsone. After dapsone had to be discontinued because of side effects, a gluten-free diet and supportive therapy controlled the disease; the AEmA and tTG antibodies became negative. Our data on 10 consecutive DH cases examined by DIF and by serum studies for AEmA and antibodies to tTG, point to frequencies of 90% DIF positive and 70% AEmA and tTG positive cases. The use of both DIF and serum tests for AEmA and tTG reveals DH cases not detected by DIF alone that respond to gluten-free diet. Findings on autoantibodies to tTG, an enzyme that metabolizes gliadin, points to a role of tTG in the immunopathology of gluten-sensitive enteropathy and helps to explain the need for a gluten-free diet in the management of DH cases. ( info) |
| We describe the case of a 53-year-old woman presenting papulous and papulovesicular lesions that were highly pruritic, localized mostly in the achromic areas of vitiligo and symmetrically distributed on the elbows, the buttocks, the shoulders and the neck. The histopathological examination performed on the elbow's lesional skin showed the presence of neutrophils and fibrin microabscesses at the tips of dermal papillae, with a few eosinophils, and small separations between the dermis and epidermis just over the infiltrate. The overlying epidermis was uninjured. The performed tests detected IgA anti-endomysium, anti-thyrogloblin, anti-smooth muscle and anti-microsomal fraction autoantibodies; DIF showed the presence of IgA granular deposits at the dermo-epidermal junction, prevalently at the tips of dermal papillae. This is the tenth case reported of an association between dermatitis herpetiformis and vitiligo. Although the two disorders both have immunological pathogeneses, we think that the topographic coexistence of both disorders is coincidental. ( info) |
| Previous studies have reported the association between celiac disease and T cell lymphoma of the intestine as well as hepatosplenic lymphoma, a specialized peripheral type of T cell lymphoma. In this report, a 66-year-old woman with dermatitis herpetiformis and biopsy-defined celiac disease developed a thyroid mass that proved to be a T cell lymphoma. A T cell lymphoma in the setting of celiac disease appears to be unique. The thyroid gland, due to its shared embryological developmental links with the gastrointestinal tract, is possibly another site of extranodal lymphoma linked to celiac disease. ( info) |
| We report a 73-year-old Japanese man with dermatitis herpetiformis which developed after diagnosis of anaplastic large cell lymphoma. The patient suffered fever, sweating, shivering, and multiple enlarged cervical lymph nodes. The diagnosis of anaplastic large cell lymphoma was confirmed by the histologic features of a biopsied cervical lymph node. The patient underwent combination chemotherapy. However, one month after the initial therapy, pruritic erythematous skin lesions with peripheral vesicles appeared on his buttocks. A skin biopsy showed subepidermal blister formation associated with polymorphonuclear and mononuclear cell infiltrates. Direct immunofluorescence examination of the area adjacent to the lesion showed granular deposits of IgA at the dermoepidermal junction. While it is well-known that dermatitis herpetiformis can develop into lymphoma, there have been only a few reports of its appearance after a diagnosis of lymphoma. This case suggests that dermatitis herpetiformis may be induced by anaplastic large cell lymphoma. ( info) |