Purpura and leg ulcers in a patient with cryoglobulinaemia, non-Hodgkin's lymphoma, and antiphospholipid syndrome.

We report a 69-year-old Caucasian female with non-Hodgkin's lymphoma who subsequently developed leg ulcers as a manifestation of the antiphospholipid syndrome. Investigations revealed a mixed cryoglobulinaemia with monoclonal IgM-kappa and antiphospholipid activity with anticardiolipin antibodies, antimitochondrial type M5 antibodies and lupus anticoagulant. Significantly increased concentration of anticardiolipin antibodies was detected in the cryoprecipitate. Our case illustrates a connection between cryoglobulinaemia and lymphoproliferative and autoimmune disorders. Both cryoglobulins and anticardiolipin antibodies could participate in the vascular damage. Cutaneous manifestations in the presence of these disorders associated with non-Hodgkin's lymphoma have not been described previously.

Speckled antinuclear antibodies in keratinocytes--what does it mean?

OBJECTIVE: Epidermal in vivo nuclear staining is occasionally noted in the lupus band test (LBT) in patients with connective tissue diseases (CTD). The exact clinical significance of this finding remains unelucidated, especially in association with a positive LBT We have reviewed the clinical and serological characteristics of patients with in vivo-bound antinuclear antibodies (ANA) in keratinocytes. METHODS: Between 1990-1999 speckled IgG staining in keratinocyte nuclei was observed in 31 LBT specimens. We had detailed clinical and laboratory data for 22/31 patients. The present study comprises 22 patients with in vivo-bound ANA (8 cases with mixed CTD (MCTD), 10 with systemic lupus erythematosus (SLE), 2 with Sjögren's syndrome (SS), one with undefined CTD and one clinically healthy mother of a child with neonatal lupus erythematosus), and 22 consecutive CTD patients (2 MCTD, 15 SLE, 5 SS) without in vivo-bound ANA. Antinuclear, anti-dsDNA and anti-extractable nuclear antigens (ENA) antibodies were determined using indirect immunofluorescence and ELISA methods. RESULTS: A significant difference (p < 0.01) in anti-RNP antibodies between patients with and without in vivo-bound ANA was observed. Consequently, there was a strong association of in vivo-bound ANA and anti-RNP antibodies (p < 0.01). In SLE patients with in vivo-bound ANA the incidence of nephropathy was significantly lower (p < 0.01), regardless of LBT positivity. In SLE patients there were no differences in LBT positivity, anti-dsDNA antibodies and complement consumption between groups. CONCLUSION: Our study implies that the presence of speckled ANA in keratinocytes in LBT may be useful in the diagnosis of MCTD and in the prognosis of renal involvement, especially in SLE patients.

Generalized acanthosis nigricans in early childhood.

Acanthosis nigricans (AN) is a disorder often accompanied by internal malignancy in adult patients, but in children it is usually benign. Generalized forms of AN, especially in childhood, are extremely rare. We report a 5-year-old boy with a 3-year history of AN who had generalized roughness and hyperpigmentation, numerous skin wrinkles, disseminated skin tags, tripe palms, and intensive pruritus. No clinical evidence of internal disorders, notably of an endocrinologic or neoplastic nature, have been detected thus far.

Toxic epidermal necrolysis following morbilli-parotitis-rubella vaccination.

We present the first reported case of toxic epidermal necrolysis (TEN) caused by morbilli-parotitis-rubella (MPR) vaccine. A 13-year-old girl developed TEN 7 days after she received live, attenuated, triple MPR vaccine. The history of drug intake and any illness was negative. At admission the patient was acutely ill with high fever. The whole body was erythematous. The epidermis was wrinkled and the Nikolsky sign was positive. Numerous erosions were present on the lips and genital region. On the seventh day of illness, the eruption involved 80% of the skin. Systemic corticosteroid therapy was not employed. The skin and mucosal defects completely epithelized by the end of the third week of illness. Mild keratoconjunctivitis sicca remained because of permanent cup cell damage.

[Study of hair in type I tricho-rhino-phalangeal syndrome]. / Etude des cheveux du syndrome trichorhinophalangien type I.

Trichorhinophalangeal syndrome has three characteristic signs: slow-growing hair, pearshaped nose with high philtrum and bradyphalangia with wedge-shaped epiphyses. We report two familial cases of type I trichorhinophalangeal syndrome. Our aim was to better identify the hair anomalies. A 22-year-old boy and his 65-year-old mother had thin sparse hair with recessed fronto-temporo-occipital hair lines. The trichogramme of the occipital region showed 52% dystrophic roots and 48% telogenic roots. Polarized light revealed monochromal hair with clear << finger-end >> rhexis. Electron scan microscopy showed thin, oval, flat or cannulated hairs and folded cuticle cells. A transverse zone in the prerhexis area had no cuticle cells and << finger-end >> fractures. Histological examination showed a normal follicle count. Sebaceous and sudoriparous glands were normal. Other characteristics of trichorhinophalangeal syndrome (facial deformity with pear-shaped nose with high philtrum, clinobrachydactylia of the fingers and toes, and radiologically wedge-shaped epiphyses) were also noted. Caryotypes were normal. We emphasize the importance of folded cuticle cells and clear rhexis leading to << finger-end >> fractures. These anomalies could serve as markers in cases with few suggestive signs.

Diffuse partial woolly hair.

We report 4 new cases presenting with the characteristics of a recently described pilar dysplasia entitled 'diffuse partial woolly hair'. Two of our cases were familial (mother and daughter), while the 2 other were sporadic. Scalp hair of all the patients exhibited two hair shaft populations intermingled throughout the scalp. The first hair type was straight, normally pigmented and of normal length, while the second type was wavy and the shafts were hypopigmented, thinner, shorter and easily pluckable. One of our patients presented with a diffuse hair thinning due to a progressive loss of wavy hairs. Under reflected light, both hair populations displayed alternating dark and bright segments. Scanning-electron microscopic analysis of straight and wavy hairs revealed comparable lesions: torsions, angular kinks, weathering as well as grooved segments alternating with the normal ones. These changes were the most severely expressed in the shortest and the waviest hairs. Taken together, the clinical and microscopic characteristics of this pilar dysplasia justify its introduction as a separate entity into the woolly hair group.