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Lymphomatoid papulosis/pityriasis lichenoides in two children.

Two children developed lymphomatoid papulosis/pityriasis lichenoides at ages 3 and 6 years. Follow-up continued for 13 years in the former patient and for 6 years in the latter. Both children now have continuing low-grade disease activity requiring in the one case topical corticosteroid therapy and in the other low-dose systemic steroid therapy. These children are reported to emphasize to pediatricians, pediatric pathologists, and hematologists that pseudolymphomatous conditions can exist in young children and do not require potent cytotoxic therapy. In both of our patients, the initial diagnosis was thought to be an aggressive lymphoma.

Child↗

Follicular and perforating disorders.

Pathologic changes associated with hair follicles include infundibular hyperkeratosis, inflammation, mucinous degeneration and transepidermal elimination of foreign material. Because of the focal nature of the pathologic changes multiple sections are often useful in establishing the correct diagnosis.

Folliculitis↗

Mucha-Habermann disease: a diagnostic possibility for prolonged fever associated with systemic and skin symptoms.

The severe form of Mucha-Habermann disease with systemic symptoms is a rarely diagnosed disease which should be considered for children with prolonged fever, impaired general condition, skin manifestations and elevated C-reactive protein concentration and/or erythrocyte sedimentation rate. Eleven cases have been described previously in children. We describe two acute episodes of this syndrome in a three-year-old child; the diagnosis was based on clinical, dermatological and histological findings. During both episodes, the fever lasted for more than one week, C-reactive protein concentration increased to more than 150 mg/l, and there was extensive lymph node enlargement. Skin eruption was initially maculopapulous, then vesiculous and finally pustulous. On skin biopsy, vasculitic changes were observed. We treated the second attack of our patient with high-dose gamma globulin; the first attack appeared to resolve itself spontaneously.

Arthritis, Juvenile↗

Vitamin B12 excretion in patients with various skin diseases.

The excretion in the urine of (58)Co after an oral dose of (58)Co vitamin B(12) given together with intrinsic factor has been found to be reduced in a number of patients with psoriasis, eczema, and other less common dermatoses. There is a correlation between the abnormality and the extent of the rash. A reduced glomerular filtration rate was found in a few of the patients in whom it was measured, and this must have been responsible, at least in part, for the reduced excretion of vitamin B(12) in these patients, but abnormal vitamin B(12) excretion also occurred in the absence of impaired renal function. Our evidence is insufficient to show whether malabsorption or increased tissue utilization of vitamin B(12) was the explanation in other cases. Certainly a number of patients had steatorrhoea, and in these it is most likely that malabsorption was the major factor. In patients without steatorrhoea a lone malabsorption of vitamin B(12) cannot be excluded. A decreased serum concentration of vitamin B(12) was found in only one of the patients.

Adult↗

Comparison of cell adhesion molecule expression in cutaneous leucocytoclastic and lymphocytic vasculitis.

AIMS: To compare the expression of the cell adhesion molecules intercellular adhesion molecule-1 (ICAM-1), ELAM-1 (E-selectin), and vascular cell adhesion molecule-1 (VCAM-1) in cutaneous leucocytoclastic and lymphocytic vasculitis. METHODS: Immunohistochemical analysis was performed on early lesional skin biopsy specimens of leucocytoclastic vasculitis (n = 14), lymphocytic vasculitis (n = 10), non-lesional skin (n = 12), and normal skin (n = 5). A standard immunoperoxidase technique was used to detect expression of ICAM-1, E-selectin, VCAM-1, and the cell markers CD11a, CD11b, CD11c, von Willebrand factor, CD3, CD68, and neutrophil elastase (NP57). RESULTS: Basal keratinocyte intercellular adhesion molecule-1 was expressed in eight (80%) cases of lymphocytic and in only one (7%) case of leucocytoclastic vasculitis, and not in non-lesional skin or control biopsy specimens from normal subjects. E-selectin was expressed on vascular endothelium in eight (57%) cases of leucocytoclastic and in seven (70%) cases of lymphocytic vasculitis. Endothelial vascular cell adhesion molecule-1 expression was seen in three (21%) biopsy specimens of leucocytoclastic and five (50%) of lymphocytic vasculitis. There were increased numbers of cells in the dermal infiltrate stained for NP57, CD11b, and CD11c in leucocytoclastic compared with lymphocytic vasculitis (p < 0.001, p = 0.013, p = 0.009, respectively); immunoreactive positive cells for CD3 and CD11a were increased in lymphocytic compared with leucocytoclastic vasculitis (p < 0.001, p = 0.011, respectively). CONCLUSIONS: These observations indicate that upregulation of adhesion molecule expression occurs in both leucocytoclastic and lymphocytic vasculitis. The different patterns of adhesion molecule expression in the two groups of vasculitis may reflect differences in the local release of cytokines. In particular, detection of intercellular adhesion molecule-1 expression by keratinocytes in lymphocytic vasculitis is consistent with an active role for mediators derived from T lymphocytes in the pathogenesis of the lesion.

Cell Adhesion Molecules↗