Acta Demato-Venereologica 98-3CompleteContent | Page 8

REVIEW ARTICLE
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Treatment of Scleroedema Adultorum Buschke: A Systematic Review
Diana MIGUEL, Sibylle SCHLIEMANN and Peter ELSNER
Department of Dermatology, University Hospital, Jena, Germany
Scleroedema adultorum Buschke is a rare skin disease,
which can be divided into 3 subtypes: classic type, oc-
curring after respiratory infections; a type lacking as-
sociation with infections; and a type associated with
diabetes. Scleroedema adultorum Buschke is charac-
terized by thickening and tightening of the skin, which
typically starts at the neck. In half of patients, spon-
taneous remission may occur. The aim of this syste-
matic review is to summarize all reported treatments
for scleroedema adultorum Buschke, based on articles
from PubMed database, using the query “scleroedema
adultorum Buschke treatment”, English and German,
published between 1970 and 2016 and documenting
adequate treatments. The results are based mainly on
individual case reports, small case series, and retro-
spective studies often reporting unsuccessful results.
Treatment options include topical as well as systemic
treatments, and physical modalities. There is a need
for randomized controlled trials and studies on long-
term outcomes after treatment.
Key words: scleroedema adultorum Buschke; treatment.
Accepted Nov 13, 2017; Epub ahead of print Nov 14, 2017
Acta Derm Venereol 2018; 98: 305–309.
Corr: Diana Miguel, Department of Dermatology, University Hospital, Er-
furter Straße 35, DE-07740 Jena, Germany. E-mail: Diana.Miguel@med.
uni-jena.de
S
cleroedema adultorum Buschke (SAB) is a rare skin
condition of unknown aetiology that was first descri-
bed by Curzio in 1752. However, its name is associated
with Abraham Buschke who reported a 46-year-old
patient developing skin hardening after an episode of
influenza in 1902. The nomenclature has been criticized,
since the disease also occurs in younger individuals
lacking sclerosis. In fact, in 209 patients with SAB,
51% were younger than 20 years old. For childhood
cases, SAB must be differentiated from scleroedema
neonatorum (1–4).
SAB is characterized by skin thickening and tighte-
ning that typically starts at the neck and spreads over
the upper part of the body, e.g. the face, scalp, shoulder,
and trunk (Fig. 1). The hands and feet are usually not
affected (5, 6). A case of SAB confined to the thighs has
also been reported (7).
SAB can be divided into 3 types:
• type 1, or “classic type”, occurs in patients who have
recently had a bacterial or viral febrile infection, usually
of the upper respiratory tract. This type tends to resolve
spontaneously within a few weeks up to 2 years.
• type 2 refers to patients without an underlying infection
as trigger factor, with a slow progressive course, and
a tendency to chronicity. This type is also associated
with paraproteinaemias with onset at a younger age.
• type 3, also named “diabetic scleroedema”, is asso-
ciated with diabetes mellitus, has a slow progressive
course and, unlike types 1 and 2, occurs more often
in men (8, 9). It is suggested that an irreversible gly-
cosylation of collagen and alterations in collagenase
activity lead to an accumulation of collagen and mucin
in the dermis (10).
There are no specific laboratory abnormalities, but the
diagnostic workup of SAB should include analysis of
anti-streptolysin O titre, haemoglobin A1c, sedimenta-
tion rate, blood count, and protein electrophoresis. There
is no racial or ethnic propensity, but women are usually
more affected with type 1 and type 2 SAB than are men
(1). Scleroedema has also been associated with internal
malignancies, e.g. insulinoma and carcinoma of the gall
bladder (11, 12).
Histologically, SAB is characterized by a normal
epidermis with an up to 4 times thickened dermis con-
taining mucopolysaccharides, as evidenced by Alcian
blue stain, in oedematous spaces between collagen fibres.
The subcutaneous fat tissue is usually replaced by coarse
collagen fibres (13).
The course of scleroderma is usually slow and rarely
fatal. Patient’s complaints include tightness, thickening,
Fig. 1. Scleroedema adultorum Buschke with extensive severe
skin thickening and solid oedema of the neck, back, shoulders
and upper arms.
This is an open access article under the CC BY-NC license. www.medicaljournals.se/acta
Journal Compilation © 2018 Acta Dermato-Venereologica.
doi: 10.2340/00015555-2846
Acta Derm Venereol 2018; 98: 305–309