[Eruptive vellus hair cysts]. / Eruptive Vellushaarzysten.

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.

[Exacerbation of Hailey-Hailey disease by infection with herpes simplex virus. Detection with polymerase chain reaction]. / Exazerbation eines Morbus Hailey-Hailey durch Infektion mit Herpes-simplex-Virus. Nachweis mittels Polymerasekettenreaktion.

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.

[Hamartoma of the sebaceous follicle. An immunohistologic analysis with cytokeratins]. / Hamartom des Talgdrüsenfollikels. Eine immunhistologische Analyse mit Zytokeratinen.

A 62-year-old man presented with a nodule 2 cm in diameter on his left cheek, which he had had since childhood. Histological examination showed a circumscribed dermal nodule composed of sebaceous lobules attached to sebaceous ducts and to an infundibular cyst-like structure connected to the epidermis. The wall was lined with squamous epithelium with a stratum granulosum. This structure was reminiscent of a sebaceous follicle. In addition, a melanocytic naevus of the compound type was found. Immunohistological investigations of the tumour with various cytokeratins revealed a pattern of expression characteristic for the mature sebaceous follicle.

[Congenital onychodysplasia (Iso-Kikuchi syndrome)]. / Kongenitale Onychodysplasie (Iso-Kikuchi-Syndrom).

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.

[Dyskeratosis congenita in monozygous twins]. / Dyskeratosis congenita bei monozygoten Zwillingen.

Dyskeratosis congenita (DC) is a very rare form of genodermatosis with variable manifestations, which mainly affects male patients. The main clinical symptoms are poikilodermia, nail dystrophy and leucoplakia; there are many other cutaneous and systemic symptoms. To avoid complications and improve the prognosis early diagnosis and regular close surveillance of the patients are important. We report on 13-year-old monozygotic twin brothers who, in addition to the typical symptoms, had increased vulnerability of the skin, scarring of the hands and atrophy of the oral mucosa as well as splenomegaly, pancytopenia with severe aplasia of bone marrow and aseptic necrosis of the hip. The two brothers had nearly synchronous clinical manifestation and progression. This paper reviews the clinical symptoms, pathogenesis, differential diagnosis and genetic aspects of DC.

Papular elastorrhexis: a variant of dermatofibrosis lenticularis disseminata (Buschke-Ollendorff syndrome)?

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.

[Herpes gestationis: immunologic and immunogenetic aspects]. / Herpes gestationis: immunologische und immungenetische Aspekte.

Herpes gestationis or pemphigoid gestationis (PG) is a bullous disease developing in association with pregnancy. It is believed to be an immunologically mediated disorder. Antibody binding by specific autoantibodies was detected in placental tissue and in the area of the hemidesmosomes and the basal membrane zone of the skin. These autoantibodies react with antigens of 180 kDa and 230 kDa relative molecular mass, which are presumably identical to the bullous pemphigoid antigen BPAG1 and BPAG2. The herpes gestationis factor (HG factor) preferentially binds to the 180-kDa antigen (BPAG2). The involvement of specific autoantibodies against adhesion molecules suggests the involvement of an aberrant immune response. Several groups have reported an association of pemphigoid gestationis to alleles of the human leukocyte antigens; HLA-B8 (class I) and HLA-DR3 and HLA-DR4 (class II), encoded on the short arm of chromosome 6. The unique feature of pemphigoid gestations is compared with other bullous dermatoses the association with the hormonal regulation during pregnancy and the presence of allogenic tissue. The hormonal regulation at the level of gene expression, possibly including gene expression of non-classic HLA class I molecules such as HLA-G, may be a cofactor in the pathogenesis of pemphigoid gestationis. This account, together with a case report, discusses the association of pemphigoid gestationis with HLA and a putative pathophysiological role of the newly described non-classical HLA molecules encoded in the MHC.

[Immunogenetic findings in herpes gestationis]. / Immungenetische Befunde bei Herpes gestationis.

A 27-year-old woman suffered from herpes gestationis in her second pregnancy. The pruricy urticarial and bullous skin lesions were exacerbated immediately after birth, and premenstrually after the onset of menstruation. The diagnosis was confirmed by the determination of anti-basement membrane antibodies of the IgG class both in the serum (herpes gestationis factor) and in lesional skin by immunofluorescence techniques. The newborn had no skin lesions and no basement membrane zone antibodies were detected. The HLA (human leucocyte antigen) typing revealed the HLA-A11/1, B18/w63, C-/-, DR4/w13, DQw3/w1 haplotype in the mother and the HLA-A3/31, B7/35, Cw7/w4, DR2/3, DQw1/w2 haplotype in the father. Comparison with the first child (HLA-A11/31, B18/35, -/Cw4, DR4/3, DQw3/w2) showed that the second child (HLA-A11/3, B18/7, -/Cw7, DR4/2, DQw3/w1) had a different HLA haplotype. The pattern of the HLA haplotype of the mother and the father seems to be important in the immunopathogenesis of herpes gestationis.

[Extra-abdominal fibromatosis. An immunohistochemical analysis]. / Extraabdominale Fibromatose. Eine immunhistochemische Analyse.

A 49-year-old patient presented with a slow-growing tumour on his back, which had been present for 10 years. Histologically an infiltrating spindle-cell tumour without cell polymorphism was found. Each cell was surrounded by collagen fibrils. Immunohistochemically the tumour cells showed positive staining with antibodies to alpha-smooth muscle actin, factor XIIIa, CD34 and vimentin. In contrast, antibodies to the intermediate filament protein desmin, glial fibrillary acid protein (GFAP), the neurofilament (NF), neuron-specific enolase (NSE), S-100 protein, carcinoembryonic antigen (CEA) and keratin protein (LU-5) revealed no staining of the spindle cells. This immunohistochemical profile supports myofibroblastic differentiation of the spindle cell in extra-abdominal fibromatosis.