Photoepicutaneous testing UV-induced protection against photoxic reactions in normal and vitiliginous skin: A clinical and histological study

The effect of erythemic UV irradiation on the phototoxic reactions caused by topical methoxsalen + UVA exposure was studied on normal skin, normal-looking skin of vitiligo patients, and vitiliginous skin. Although only slight histological changes were detectable 9 days after irradiation with 5 MED of erythemic UV, this pre-irradiation did induce protection against photoxic reactions in all skin types. This protection was clinically equal in all skin types; the slight differences were not statistically significant. Histological evaluation, however, showed a most conspicuous protective effect on vitiliginous skin. In all skin types the influence of UV pre-irradiation was confined to epidermal protection; the dermal phototoxic changes were unaffected.     

Mitotic Langerhans cell as a possible sign of activation in ichthyosis

The fine structure of Lagerhans cells (LC) in four rare types of ichthyosis, namely recessive ichthyosis congenita type II, III and IV and ichthyosis hystrix Curth-Macklin was examined. Signs of LC activation were observed in eight of 21 patients. In IC type IV, the rare occurrence of a mitotic LC was observed. It is possible that LCs are secondarily activated in keratinization disorders.     

Light and electron microscopy of psoriatic skin before and during retinoid (Ro 10–9359) and retinoid-PUVA treatment

High patients with psoriasis (pustular, erythrodermic or severe plaque type) were treated sucessfully with retinoid (Ro 10-9359) or with retinoid followed by PUVA (RePUVA). The histological changes in recovering skin were examined during 14 weeks. Treatment resulted in normalization of keratinocyte differentiation with fine structural changes suggesting decreased metabolic activity and a reduction of mitoses. There was an increase in the number of keratohyalin granules and of tonofilaments and desmosomes and a decrease in leukocytes in the epidermis. The different cell organelles were studied in detail, but the exact mechanism of retinoid action remains unsolved. Although the treatments were clinically highly effective, total normalization of the histological and electron microscopical picture was not achieved.     

Histological and ultrastructural study of a family with erythrokeratodermia progressiva symmetrica

We have examined a family with 4 members in three succeeding generations suffering from a severe keratinization disorder. The clinical phenotype, with symmetric plaques on the extremities, corresponded to erythrokeratodermia progressiva symmetrica. It was manifested at birth, however, and in addition to the hyperkeratotic plaques, follicular hyperkeratosis was also observed. Electron microscopy revealed multiple morphological changes such as myelinated membrane structures, or needles, which were similar to those occurring in ichthyotic disorders and tyro sinemia, as well as in harlequin fetuses, all of which were excluded clinically or biochemically in our patients.     

Autosomal recessive congenital ichthyosis in Sweden and Estonia: clinical,genetic and ultrastructural findings in eighty-three patients

Congenital (non-bullous) ichthyosis is a rare group of keratinizing disorders which can be tentatively subclassified based on clinical criteria, analysis of transglutaminase 1 gene mutations and electron microscopy of epidermis. We studied 83 patients who were all on topical therapy and in 16 cases also on oral retinoids. Three main groups of patients were distinguished: (A) those with transglutaminase 1 gene mutations (n=44), (B) those without transglutaminase 1 gene mutations showing a coarse, generalized scaling (n=19), and (C) those without transglutaminase 1 gene mutations showing only fine or focal scaling (n=20). On clinical scoring, patients in group A were more hyperkeratotic and less erythematous than those in group B (p < 0.05). Anhidrosis was recorded in nearly all patients (> or = 80%), but ectropion and a collodion phenotype at birth were more common in group A versus other groups. Ultrastructurally, a high frequency of type I (Anton-Lamprecht's classification) was found in all three groups (37-63%), 20 cases of type II in group A and a few cases of types III and IV in groups B and C, respectively. In conclusion, transglutaminase 1 gene mutation is a major cause of congenital ichthyosis in Sweden and Estonia, and is often associated with severe scaling and ultrastructural type II in corneocytes. The transglutaminase-unrelated cases are more heterogeneous, probably reflecting a more varied aetiology.     

Plasmacellular Angiodermatitis and Hypertrophy of Extremities

Two males with enlargement of one upper extremity, deep purple in color, and one female with a long, deep-red stripe along the lower extremity were found to have an increased number of tiny vessels in the dermis with dense round-cell infiltration consisting mainly of plasmacells. There were no signs of arteriovenous shunts, venous malformations or diseases associated with plasmacells. Polycythemia and thrombocythemia were excluded. Some immunological abnormalities were found in all cases. This condition seems to be self-healing.     

New progeroid disorder

We report on a 10-year-old Caucasian male with a prematurely aged appearance, delayed bone maturation and dental development, pronounced acro-osteolysis with brachydactyly, and distinctive cutaneous findings including hard, confluent skin lesions with some clinical and histologic resemblance to those of juvenile hyaline fibromatosis (JHF). He also had hyperopia, sensorineural hearing loss, and elevated TSH. Linear growth and intellectual functions were normal. We believe that this patient represents a new progeroid disorder. Am. J. Med. Genet. 69:182–187, 1997. © 1997 Wiley-Liss, Inc.     

Hyperpigmentation and Hypopigmentation of the Skin after Long Term PUVA Therapy

An electron microscopic study was performed to demonstrate the pathological changes induced by long term PUVA treatment in recalcitrant psoriasis. Three patients developed mottling (hyperpigmentation and hypopigmentation) during two to three years of treatment. Three different types of morphological changes were found: disarrangement of keratinocytes, clustering and stimulation of melanocytes and homogenization of papillary dermis. Furthermore, the superficial blood vessels were loaded with the same type of amorphous granular substance. These changes might be specific to PUVA treatment or they might occur only in patients with previous treatment with, e.g., arsenic, methotrexate, anthralin + UVB or a combination of these.