SCLEREDEMA ADULTORUM OF BUSCHKE – CASE REPORT

  • Ana Brinca Interna do Internato Complementar de Dermatologia e Venereologia/Resident, Dermatology and Venereology
  • Hugo Oliveira Assistente Hospitalar de Dermatologia e Venereologia/Consultant, Dermatology and Venereology
  • Margarida Gonçalo Chefe de Serviço de Dermatologia e Venereologia/ Consultant Chief, Dermatology and Venereology
  • Óscar Tellechea Chefe de Serviço; Professor Doutor de Dermatologia e Venereologia/ Consultant Chief, Professor of Dermatology and Venereology
  • Américo Figueiredo Director de Serviço; Professor Doutor de Dermatologia e Venereologia dos Hospitais da Universidade de Coimbra/ Head of Dermatology Department; Professor of Dermatology and Venereology of Coimbra University Serviço de Dermatologia, Hospitais da Universidade de Coimbra, EPE, Coimbra, Portugal

Abstract

Scleredema adultorum of Buschke (SAB) is a rare connective tissue disease characterized by non-pitting induration of the skin. Histologically there is thickening of the dermis with large collagen fibers separated by deposits of mucopolysaccharids.
A 51-year-old male patient, with poorly controlled diabetes mellitus, progressively developed in a 5-year period, a di- ffuse cutaneous infiltration with ill-defined limits on the posterior neck, upper half of the trunk and proximal limbs, with marked skin stiffness and a significantly reduced mobility of the shoulder girdles and neck.

The diagnosis of SAB was confirmed by histological examination. Other complementary exams did not reveal significant changes, allowing the diagnosis of the third subtype of SAB - diabeticorum. The patient was treated with phototherapy, initially with UVA1 without any benefit, and later with an experimental therapeutic modality of PUVA1 with significant clinical improvement, allowing the patient to dress himself.

KEYWORDS – Scleredema Adultorum; Diabetes Mellitus; Phototherapy; PUVA Therapy.

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Published
2011-09-28
How to Cite
Brinca, A., Oliveira, H., Gonçalo, M., Tellechea, Óscar, & Figueiredo, A. (2011). SCLEREDEMA ADULTORUM OF BUSCHKE – CASE REPORT. Journal of the Portuguese Society of Dermatology and Venereology, 69(3), 447. https://doi.org/10.29021/spdv.69.3.82
Section
Case Reports

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