1/198. Dermal vessels in acrodermatitis chronica atrophicans. An ultrastructural investigation of two patients suffering from acrodermatitis chronica atrophicans revealed in the small dermal vessels swelling of the endothelial cells, material of probably plasmatic origin accumulated in the subendothelial area, and sleeves of basement membrane-like material on a concentric perivascular layout. ( info) |
2/198. Breaking the relentless course of Hallopeau's acrodermatitis by dapsone. We report the case of a 74-year-old man who suffered for four years from recurrent generalized pustular eruptions associated with acrodermatitis continua of Hallopeau (ACH). Initial therapy using topical corticosteroids, tars and PUVA failed to improve the acral lesions. The disease was also unresponsive to systemic therapy using corticosteroids, methotrexate, etretinate, acitretine and cyclosporin A. Serious side-effects were experienced with these drugs. A marked improvement of the generalized pustules was observed under treatment combining etretinate and methotrexate, without having any effect on the acropustulosis. During hospitalization, a topical tar therapy cleared the pustular rash. Again no effect was observed on the acral lesions. Disulone (DDS) therapy, initiated at a dose of 200 mg/day, completely cleared ACH after 4 weeks. The disease relapsed when the dose was reduced to 100 mg/day. Increasing the DDS to 150 mg/day was rapidly followed by a new clearance of the acral lesions. After a 3-month treatment, the patient has remained lesion free and no DDS-related side-effects have occurred. In conclusion, sulfones may be considered as an alternative therapeutic option in drug-resistant ACH. ( info) |
3/198. Bazex syndrome mimicking a primary autoimmune bullous disorder. Bazex syndrome is a paraneoplastic condition that is most frequently associated with squamous cell carcinoma of the upper aerodigestive tract. The lesions affect acral areas of the skin, including hands, feet, ears, nose, and, to a lesser extent, elbows and knees. Lesions mimic psoriasis and dermatitis. paronychia and nail dystrophy are frequent. Bullous lesions have been reported only rarely. We report a patient with Bazex syndrome with predominantly bullous lesions that mimicked a primary autoimmune bullous disorder. ( info) |
4/198. Psoriasiform acral dermatitis: a peculiar clinical presentation of psoriasis in children. Recently an unusual chronic dermatosis, considered a new clinical entity and closely resembling psoriasis, has been described in the literature under the term psoriasiform acral dermatitis (PAD). It is characterized by cutaneous involvement of the digits without nail dystrophy. We describe three young patients, ages 6 to 8 years, in whom this condition was associated with psoriasis. Two children were affected by psoriasis vulgaris, while the third had a palmoplantar psoriasis. All laboratory investigations performed were within normal limits. skin biopsy specimens taken from the fingers of two patients revealed the pathologic features of subacute spongiotic dermatitis. Histologic examination of a biopsy specimen taken from an erythematous squamous patch confirmed the clinical diagnosis of psoriasis in two patients. The dermatitis showed a fluctuating course in all three patients, with only a moderate to strong improvement with therapy with calcipotriol ointment (50 microg/g). During follow-up, two patients experienced marked spontaneous, persistent improvement, while the disease slightly worsened in the third. The children had features similar to those described in PAD, but were also suffering from psoriasis. Whether PAD is a distinctive entity or just a clinical manifestation of psoriasis in children is still an open question. We strongly believe this latter hypothesis, although further studies are needed to confirm it. ( info) |
| acrodermatitis enteropathica is characterized by eczematous and scaly plaques on the face, scalp, acral, and anogenital regions. In addition to typical lesions, unusual prominent vesiculobullous lesions are also described. We report a full-term, 9-month-old boy who has acrodermatitis enteropathica and Pseudomonas sepsis. In this patient there were clinical findings of sepsis and eczematous vesiculobullous lesions on the periorificial and acral areas. serum zinc level was extremely low. pseudomonas aeruginosa was identified in cultures of blood and fluid which was aspirated from the bullous lesions. After oral zinc sulfate and intravenous antibiotic treatment his condition improved within 2 weeks. ( info) |
6/198. Gianotti-Crosti syndrome presenting as lichenoid dermatitis. Papular acrodermatitis of childhood (Gianotti-Crosti syndrome) is an uncommon, self-limited disease characterized by an erythematous papular eruption symmetrically distributed on the face and limbs and mild lymphadenopathy, thought to be of viral origin. The histopathologic findings are nonspecific and include focal parakeratosis, mild spongiosis, superficial perivascular infiltrate, papillary dermal edema, and extravasated red blood cells. Interface changes with some basal vacuolization may be present, but are not a conspicuous feature. We present a 2 1/2-year-old boy with multiple papules and plaques on the face and extremities and cervical lymphadenopathy. Histopathologic analysis showed compact orthokeratosis, focal parakeratosis, hypergranulosis, psoriasiform epidermal hyperplasia, and a dense lichenoid lymphohistiocytic infiltrate with extensive exocytosis of mononuclear cells. Immunoperoxidase staining with CD 1 a revealed clusters of langerhans cells in the epidermis and in the papillary dermis. In view of the clinical findings, a diagnosis of Gianotti-Crosti syndrome was made. Although there are a few reports describing a lichenoid pattern of infiltration in Gianotti-Crosti syndrome, this histologic pattern is not widely known. This case is presented to illustrate the fact that Gianotti-Crosti syndrome can present as lichenoid dermatitis, and, especially in children, should be added to the differential diagnoses of lichenoid infiltrates. ( info) |
7/198. Acrokeratosis paraneoplastica (Bazex syndrome) with adenocarcinoma of the colon: report of a case and review of the literature. Acrokeratosis paraneoplastica is a rare disease and is uncommon even in patients with upper aerodigestive tract cancer. We report a 63-year-old man with a 1-month history of numerous pruritic lesions and vesicles on both feet. Although he had received local therapy, progressive dense scale formation involving both palms and both soles was found. colonoscopy was performed because of hematochezia, and it revealed an early colon cancer. After the resection of the cancer, the skin lesions began to fall off dramatically. To the best of our knowledge, there is no report of acrokeratosis paraneoplastica associated with colon cancer in the literature. This is the first case report of acrokeratosis paraneoplastica associated with early colon cancer. ( info) |
8/198. Necrolytic acral erythema associated with hepatitis c: effective treatment with interferon alfa and zinc. BACKGROUND: Necrolytic acral erythema is a recently described necrolytic erythema that is unique in its exclusive acral location and strong association with hepatitis c. observation: We report the first case of necrolytic acral erythema in the united states. The patient is a 43-year-old black woman who presented with a 4-year history of tender, flaccid blisters localized to the dorsal aspect of her feet. serum zinc and glucagon levels were normal. serum antibodies were positive for hepatitis c, and a liver biopsy specimen showed chronic hepatitis. She was successfully treated with interferon alfa-2b and zinc. We review all previously reported cases. CONCLUSIONS: Necrolytic acral erythema is a distinct entity. In a review of the literature, most patients were between 35 and 55 years of age, although 1 patient was 12 years old. Five of 8 patients were female. Four of 7 patients described previously were treated with variable success using oral zinc sulfate and amino acids, whereas 2 were successfully treated with interferon alfa. All patients were infected with hepatitis c. Necrolytic acral erythema appears to be a skin disorder linked to infection with hepatitis c virus that responds to treatment with interferon alfa and oral zinc. ( info) |
| Gianotti-Crosti syndrome (GCS) is a distinctive, self-limiting acropapular or papulovesicular eruption. Typical clinical presentation is that of a monomorphic, nonpruritic eruption most commonly involving the face, neck, buttocks, and extremities. It tends to occur in childhood, though infrequently it has been reported in adults. We report GCS occurring in an infant following immunization. ( info) |
| An acrodermatitis enteropathica-like syndrome occurred as a result of severe bowel disease and the use of zinc-deficient hyperalimentation regimens. Emphasis is placed on early and adequate zinc replacement to effectively prevent or reverse this entity. ( info) |