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Indian J Dermatol Venereol Leprol. 2018 Oct 4. doi: 10.4103/ijdvl.IJDVL_288_17. [Epub ahead of print]

Scleredema of Buschke associated with lichen sclerosus: Three cases.

Author information

1
"Mario Aresu" Department of Medical Science, Section of Dermatology, University of Cagliari, Cagliari, Italy.
2
Clinic of Dermatology, Ss. Cyril and Methodius University, Skopje, Republic of Macedonia.
3
Dermatology Clinic, AOU Cagliari, Cagliari, Italy.

Abstract

Scleredema adultorum of Buschke is a rare fibromucinous, scleroderma-like connective tissue disease most commonly found in a post-infectious setting or linked to hematological disorders or diabetes. Lichen sclerosus et atrophicus is an autoimmune condition only in 2.5% of cases localized exclusively at an extragenital site, occurring in up to 34% of patients in association with other autoimmune diseases such as vitiligo, thyroid disorders, alopecia areata, lichen planus, morphea, pernicious anemia and systemic lupus erythematosus. In particular, a stronger link with an autoimmune background in lichen sclerosus et atrophicus has been observed in women who showed higher prevalence for autoimmune conditions and circulating autoantibodies. Literature reveals a genetic susceptibility linked to specific HLA types. We report three patients who developed lichen sclerosus et atrophicus superimposed on skin involved by scleredema adultorum of Buschke. Although the association of lichen sclerosus et atrophicus with scleredema adultorum of Buschke could be coincidental, both diseases could be considered part of the spectrum of sclerodermoid disorders with common underlying pathogenetic mechanisms; which could explain the sequential or simultaneous occurrence of both lesions in our patients.

KEYWORDS:

Bullous lesions; bullous lichen sclerosus et atrophicus; lichen sclerosus et atrophicus; scleredema adultorum

PMID:
30289118
DOI:
10.4103/ijdvl.IJDVL_288_17
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