Resident Rounds Part III: Scleromyxedema: A Rare Disorder Associated With a Monoclonal Gammopathy
May 2014 | Volume 13 | Issue 5 | Features | 619 | Copyright © May 2014
Jenny A. Mandell MD, Jaimie B. Glick MD, and Ravneet Ruby Kaur MD
Department of Dermatology, SUNY Downstate Medical Center, Brooklyn, NY
Scleromyxedema is a rare primary cutaneous mucinosis affecting middle-aged adults. It is characterized by dermal mucin deposition
with increased collagen in the skin and internal organs. We report a case of a 72-year-old man with classic skin findings of scleromyxedema
as well as a monoclonal gammopathy.
A 72-year-old man originally from Grenada presented to
the dermatology clinic at SUNY Downstate Medical
Center for evaluation of a generalized rash. The patient
reported a three year history of a progressive rash, with
increased thickening of his skin, and pruritus. He also noted
swelling of his hands and fingers with decreased range of motion
and a left eyelid ptosis. He was treated by an outside dermatologist
with topical triamcinolone and halobetasol for one
year without improvement based on a biopsy report showing
granuloma annulare. Review of systems was otherwise negative.
His medical history was significant for hypertension and
glaucoma. His additional medications included pantoprazole,
amlodipine, travanoprost, atenolol, and aspirin.
Physical exam revealed numerous waxy, erythematous
2-3mm papules coalescing into thickened hyperpigmented
plaques covering much of the neck, chest, back, and extremities.
Indurated skin folds were most evident in the lateral
trunk, axillae, and upper arms consistent with the "Shar-Pei
sign". (Figure 1). In several areas, the papules formed linear
arrays. His hands were sclerotic with swelling of all digits.
The patient had a left eyelid ptosis, loss of eyebrow hair bilaterally
(madarosis), and a leonine facies (Figure 2). Two 4 mm
punch biopsies of the chest and back revealed a proliferation
of plump fibroblasts interposed between slightly thickened
collagen bundles with hints of mucin deposition (Figures 3a
and 3b). Immunostains for Factor XIIIa and CD34 were positive
while S-100 and CD1a were negative, consistent with a
diagnosis of scleromyxedema (Figure 4). Serum and urine electrophoresis demonstrated an IgG-kappa monoclonal
gammopathy. The patient was referred to hematology/oncology
for further management.
Scleromyxedema is an idiopathic primary cutaneous mucinosis
affecting middle-aged adults. It is characterized by
dermal mucin deposition with increased collagen in the skin
and internal organs. Patients present with waxy papules and
plaques with sclerodermoid changes.1 Patients typically have
a monoclonal gammopathy and systemic manifestations. The
significance of the monoclonal gammopathy is unclear and
paraprotein levels do not always correlate with disease severity
or clinical course.2 Furthermore, most patients do not go
on to develop multiple myeloma. Internal manifestations vary
and can include muscular, neurologic, gastrointestinal, pulmonary
and cardiovascular complications. The most common
extracutaneous manifestation of scleromyxedema is dysphagia.3 Treatment is often unsuccessful and has included a range
of agents such as melphalan, systemic steroids, phototherapy,
intravenous immunoglobulins, plasmapheresis, electron
beam radiation, and various chemotherapies. More recently,
combinations of thalidomide and dexamethasone, lenalidomide,
bortezomib, and autologous hematopoietic stem cell
transplantation have been tried.4-7 Spontaneous resolution
has rarely been reported.2
Histopathology Photos by Qiang Xie MD.