Background: Buschke-Ollendorff syndrome is an autosomal dominant disorder clinically characterized by the appearance of disseminated white papules and osteopoikilosis. Histologically most cases show normal collagen and increased elastic tissue. Abortive forms of Buschke-Ollendorff syndrome are described, which show characteristic skin involvement, absence of skeletal changes and decreased elastic tissue. Papular elastorrhexis is characterized by nonfollicular, white papules, decreased elastic tissue, no genetic inheritance and no osteopoikilosis. Objective: Is papular elastorrhexis a new entity or an abortive form of Buschke-Ollendorff syndrome? Methods: We examined three members of one family (brother, sister and mother) presenting with nonfollicular, distributed, white papules on the trunk and extremities. Skin biopsies were examined by histological and electron-microscopic methods. Results: The histological and electron-microscopic examinations of skin biopsies showed decreased, fragmented elastic fibers and normal collagen. By X-ray examination, no osteopoikilosis was found. The family presented here supports a genetic background of the disease. Conclusion: The clinical appearance with the absence of osteopoikilosis and the histological findings of our cases suggest the diagnosis of papular elastorrhexis. Papular elastorrhexis however was reported to be nonfamilial. Because of the genetic background found here we believe that papular elastorrhexis is an abortive form of Buschke-Ollendorff syndrome. Summarizing our data and reviewing the literature, we suggest that connective tissue nevi with the most prominent alterations in the elastic tissue should be classified under the term elastic tissue nevi.

Eruptive vellus hair cysts are rare epidermal cysts with vellus hairs, which appear in children or young adults. A 21-year-old patient is reported who developed asymptomatic comedo-like papules on his face, neck and chest. Histopathologically, epidermoid cysts with a fine stratum granulosum, laminated keratinous material and numerous vellus hairs were found in the mid-dermis. Evaluation of immediate family members revealed that the patient's father was similarly affected. Eruptive vellus hair cysts and steatocystoma multiplex are clinically similar, but have distinctive histopathological features and should be regarded as distinct entities.

A 49-year-old man suffered from Hailey-Hailey disease for several years. The patient presented with an acute exacerbation of the disease, which did not respond to oral treatment with high doses of glucocorticosteroids. A skin biopsy was taken and the histological examination indicated a viral infection. Herpes simplex virus was confirmed by electron microscopy (negative staining) and polymerase chain reaction (PCR). The PCR presents a sensitive and effective molecular-biological method for the diagnosis of viral infections.

A 1-year-old girl shows changes of the toenails since birth. There are no associated anomalies typical for genodermatoses, nor is there a family history of this disorder. Physical examination shows missing or incomplete toenails. In addition a shortening of the second toe on both sides and the third toe on the left side is found. The second and third toe on the left side shows incomplete syndactylia. The fingers and fingernails are all normal. Isolated congenital onychodysplasia of the toenails has not been previously reported.

No abstract

No abstract

No abstract

No abstract