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[Keratosis follicularis spinulosa decalvans (Siemens' syndrome) associated with other abnormalities].
Med Cutan Ibero Lat Am 1985; 13(2):175-81MC

Abstract

Keratosis follicularis spinulosa decalvans (ichthyosis follicularis or Siemens's syndrome) is considered a general form of keratosis pilaris decalvans. Localized types are keratosis pilaris atrophicans and atrophoderma vermicularis. A case of this unusual process is presented. Clinical, histological and scanning electron microscopic studies of the hair were performed. Clinically, a generalized hypotrichosis with hyperkeratotic follicular plugs is observed; especially in the scalp and the eyebrows. Other interesting clinical findings were cutis hyperelastica, gingival hypertrophy, mongoloid palpebral fissures, big pinnae and clinodactyly of the 5th finger. From the histological point of view we observed follicular plugging, dystrophic pilosebaceous follicles, absence of sebaceous glands, perifollicular fibrosis and minimal lymphomonocytic infiltrate. Scanning electronmicroscopy shows a brittle hair with cuticular abnormalities. Siemens's syndrome can be considered a specific pilosebaceous dysplasia because the absence or hypoplasia of sebaceous glands; which produces follicular hyperkeratosis and pilar atrophy with perifollicular fibrosis and alopecia.

Authors

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Pub Type(s)

Case Reports
English Abstract
Journal Article

Language

spa

PubMed ID

3900603

Citation

Domenech, P, et al. "[Keratosis Follicularis Spinulosa Decalvans (Siemens' Syndrome) Associated With Other Abnormalities]." Medicina Cutanea Ibero-latino-americana, vol. 13, no. 2, 1985, pp. 175-81.
Domenech P, Ferrando J, Corretger M, et al. [Keratosis follicularis spinulosa decalvans (Siemens' syndrome) associated with other abnormalities]. Med Cutan Ibero Lat Am. 1985;13(2):175-81.
Domenech, P., Ferrando, J., Corretger, M., Torras, H., Valls, X., & González, A. (1985). [Keratosis follicularis spinulosa decalvans (Siemens' syndrome) associated with other abnormalities]. Medicina Cutanea Ibero-latino-americana, 13(2), pp. 175-81.
Domenech P, et al. [Keratosis Follicularis Spinulosa Decalvans (Siemens' Syndrome) Associated With Other Abnormalities]. Med Cutan Ibero Lat Am. 1985;13(2):175-81. PubMed PMID: 3900603.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - [Keratosis follicularis spinulosa decalvans (Siemens' syndrome) associated with other abnormalities]. AU - Domenech,P, AU - Ferrando,J, AU - Corretger,M, AU - Torras,H, AU - Valls,X, AU - González,A, PY - 1985/1/1/pubmed PY - 1985/1/1/medline PY - 1985/1/1/entrez SP - 175 EP - 81 JF - Medicina cutanea ibero-latino-americana JO - Med Cutan Ibero Lat Am VL - 13 IS - 2 N2 - Keratosis follicularis spinulosa decalvans (ichthyosis follicularis or Siemens's syndrome) is considered a general form of keratosis pilaris decalvans. Localized types are keratosis pilaris atrophicans and atrophoderma vermicularis. A case of this unusual process is presented. Clinical, histological and scanning electron microscopic studies of the hair were performed. Clinically, a generalized hypotrichosis with hyperkeratotic follicular plugs is observed; especially in the scalp and the eyebrows. Other interesting clinical findings were cutis hyperelastica, gingival hypertrophy, mongoloid palpebral fissures, big pinnae and clinodactyly of the 5th finger. From the histological point of view we observed follicular plugging, dystrophic pilosebaceous follicles, absence of sebaceous glands, perifollicular fibrosis and minimal lymphomonocytic infiltrate. Scanning electronmicroscopy shows a brittle hair with cuticular abnormalities. Siemens's syndrome can be considered a specific pilosebaceous dysplasia because the absence or hypoplasia of sebaceous glands; which produces follicular hyperkeratosis and pilar atrophy with perifollicular fibrosis and alopecia. SN - 0210-5187 UR - https://www.unboundmedicine.com/medline/citation/3900603/[Keratosis_follicularis_spinulosa_decalvans__Siemens'_syndrome__associated_with_other_abnormalities]_ L2 - http://www.diseaseinfosearch.org/result/3982 DB - PRIME DP - Unbound Medicine ER -