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Connective tissue nevusD23.L
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.
Congenital malformation of the corial connective tissue, usually localized in the sacral region. Occurs either in isolation or as a partial symptom of Pringle-Bourneville phacomatosis or Buschke-Ollendorf syndrome. No malignant development.
ClassificationThis section has been translated automatically.
Solitary collagen nevus (usually spontaneous occurrence)
Large nodular disseminated connective tissue nevus (familial occurrence-primarily localized on the trunk)
Multiple occurring connective tissue nevi (familial cutaneous collagenoma; OMIM 115250)
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. Am 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
- Yang Q et al (2022) Connective tissue nevus-Isolated collagenoma. Skin Res Technol 28:880-881.
- Zelger B (2002) Connective tissue tumors. Recent Results Cancer Res 160: 343-350