Synonym(s)
Cobblestone nevus; Collagen nevus; collagenous nevus lumbosacralis; Collagen rich connective tissue nevus; connective tissue nevus; Lumbosacral connective tissue nevus; Lumbosacral nevus; Paving stone nevus; Shagreen stain
DefinitionThis section has been translated automatically.
In the sacrum localized congenital malformation of the corial connective tissue. Either isolated or occurring as a partial symptom of Pringle-Bourneville phacomatosis or Buschke-Ollendorf syndrome. No malignancy development.
ClassificationThis section has been translated automatically.
- Solitary collagen nevus (mostly spontaneous occurrence)
- Large-nodular disseminated connective tissue nevus (familial occurrence-predominantly located on the trunk)
- Multiple connective tissue nevi (Familial cutaneous collagenoma; OMIM 115250)
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LocalizationThis section has been translated automatically.
The solitary form is almost exclusively located in the lumbosacral region. In principle, however, every bodily cell can be affected.
Clinical featuresThis section has been translated automatically.
Multiple, skin-coloured or whitish, symptomless, grouped standing or confluent to larger plaques (cobblestone-like aspect), flat raised nodules, plaques or knots of 0.2-5.0 cm in diameter.
HistologyThis section has been translated automatically.
Increased, densely packed collagenous connective tissue with clumpy collagen fibres. Reduced and altered elastic fibre texture, sometimes intertwined like a band. The changes are usually discreet and can only be assessed in comparison to the normal dermis.
DiagnosisThis section has been translated automatically.
The clinical picture in typical localization is diagnostic.
Differential diagnosisThis section has been translated automatically.
Connective tissue nevi of different genesis.
TherapyThis section has been translated automatically.
No therapy necessary. Excision if necessary.
LiteratureThis section has been translated automatically.
- Cesinaro AM (2003) "Connective tissue nevus" and a serendipitous S-100 discovery. On J Dermatopathol 25: 86-87
- el Fekih N et al (1993) A case for diagnosis: connective tissue nevi of the skin (hamartoma)]. Ann Dermatol Venereol 120: 639-641
- Martelli H et al (1994) Congenital soft tissue dysplasias: a morphological and biochemical study. Pediatr Pathol 14: 873-894
- Roth AM, Keltner JL (1993) Linear nevus sebaceous syndrome. J Clin Neuroophthalmol 13: 44-49
- Zelger B (2002) Connective tissue tumors. Recent Results Cancer Res 160: 343-350
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Incoming links (8)
Chagrin leather skin; Collagenous nevus lumbosacralis; Connective tissue nevus; Epidermolysis bullosa dystrophica albopapuloida; Lumbosacral nevus; Nevus, cobblestone nevus; Paving stone nevus; Shagreen stain;Outgoing links (4)
Buschke-ollendorf syndrome; Connective tissue nevus; Excision; Pringle-bourneville phacomatosis;Disclaimer
Please ask your physician for a reliable diagnosis. This website is only meant as a reference.
Prof. Dr. med. Peter Altmeyer