Incontinentia pigmenti (Bloch-Sulzberger syndrome): multisystem disease observed in two generations.

Incontinentia pigmenti is a rare genodermatosis that most commonly involves the skin, eyes, teeth, and central nervous system. We describe it in two generations. Two twin girls were observed at birth with a characteristic whorling pattern of erythema, patch, and plaques, and were later found to have dental anomalies and eye findings consisting of black pigment granules around the macula and optic nerve head. One child also had strabismus, the most common eye change, and an atrial septal defect. A scarring scalp alopecia was evident in both twins and their mother, who also had typical dental findings.

Progressive hemifacial atrophy: ten-year observation of a case.

The authors describe the case of a 14-year-old girl who experienced progressive hemifacial atrophy at the age of three-and-a-half years. The patient's early age at appearance of the condition resulted not only in atrophy of skin, subcutaneous tissue, and muscles, but also in very severe deformation of the right side of the face due to disorders in growth of cartilaginous and osseous tissue. Changes in her brain case and craniofacial skeleton included enophthalmos, underdevelopment of the eyelids, nose, hard plate, and dental process of the jaw including the premolar teeth, and a lack of the second molar. These changes caused an irregular line of bite. The differential diagnosis excluded hemifacial microsomy and sclerodermal hemifacial atrophy.

[Humoral indices of the inflammatory process in psoriasis]. / Wskazniki stanu zapalnego w luszczycy.

Immune complexes were determined in 104 patients with psoriasis, aged between 19 and 55 years. Duration of the disease, extension of lesions, and effects of infections preceding skin eruptions were considered. The studies showed the activation of immunological mechanism in psoriasis manifested by the increase in IgG and IgA levels, unchanged IgM levels, significantly more frequent negative values of IgD, significant increase in complement C4 component levels in all forms of the disease, and C3 activation in the acute and generalized chronic form. A significant increase in alpha 2 Mg was noted independently of the evolution and extension of skin lesions. An increase in alpha 1 AT was noted only in the initial phase of skin eruptions. An increase in Hp, Cr, CRP, and CIC was noted only during exacerbations. CIC were noted most frequently in postinfection psoriasis.

[Treatment of crural varicose with topical dressings of "synthetic skin"]. / Leczenie zylakowatych owrzodzen podudzi opatrunkiem ze "sztucznej skóry".

In Dermatological Department of the Lublin Medical Academy 22 patients with crural varicose ulcerations have been treated with topical Codogard dressings. Before this treatment the patients were given some antiinflammatory and vasodilatatory drugs. The treatment lasted from 4 to 12 weeks (mean 6). In 9 patients complete healing was reported (they had small ulcerations). In 10 cases the improvement was evident, in 2 cases the treatment was discontinued. The obtained results point to clinical usefulness of Codogard, as the drug is easy to be applied and it may play a significant role to outpatients.

[Changes in various apolipoprotein levels in psoriasis]. / Zachowanie sie niektórych apolipoprotein w luszczycy.

In 104 patients with common psoriasis the patterns of apolipoproteins A and B were studied in serum depending on the course of the disease, extent of skin changes and infection preceding the appearance of eruptions. The highest concentrations of apo A and apo B were found in the group with generalized psoriatic changes independent of the activity of the disease.

Mast cells in the initial stages of psoriasis.

Mast cells were counted in 122 patients with psoriasis and 80 healthy volunteers, at different times after scratch from 30 min up to 14 days. Koebner's phenomenon was induced in 74% of patients with active disease (63/85) and in 37.5% with stationary psoriasis (6/16). Mast cells were enumerated also in the skin of ten healthy controls, and their number on the inner aspect of the arm was found to range from 16 to 21 (mean, 17.6 +/- 1.65). Scarification increased the number of skin mast cells both in psoriatics and normal controls. The significant difference was noticed from the 4th day on after scratch, reaching a peak at days 14, simultaneously with the appearance of Koebner's phenomenon. In mature psoriatic lesions the number of mast cells was significantly lower than in newly formed psoriatic lesions, and it decreased progressively with effective anthralin treatment. It is concluded that mast cells play a role in skin response to trauma. In contrast to normal wound healing, their number is steadily increasing at the time of formation of the earliest psoriatic lesions.