Scleredema — Rare skin manifestation in diabetic

Abstract

Scleredema is a connective tissue disorder of unknown etiology.
A 44 years diabetic, obese female patient, admitted with edema feet, erythematous, indurated hyper pigmentation with ascending progression to trunk which rapidly transformed to painless dark pigmented blister and hard non-pitting edema of anterior abdominal wall. In a week time. She was managed with broad spectrum antibiotics, was aggressively diuresed along with albumin infusion. Her skin biopsy was done which revealed scleredema. She had poor LV function due to volume overload or cardiomyopathy. After initial improvement on 14 day, she suddenly developed fulminant septicemia. Within hours, she displayed multi organ dysfunction and the heart and kidney which were already delicately balanced, decompensated. There was intractable arrhythmias and severe LV dysfunction, renal failure and ischemic hepatitis following severe hypotension.
Scleredema in diabetic subjects, can be an ominous sign for producing fulminant multiorgan dysfunction and can present as cardiomyopathy, myocarditis, liver dysfunction etc., and these patients are more prone to infections or may be vice versa. Scleredema can be considered as a marker of fulminant illness in immunocompromised individuals. There is a need for treatment protocols or research into this a rather enigmatic but rare illness.

Full text

Southeast Asian Journal of Case Report and Review 2021;8(1):26–29
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Southeast Asian Journal of Case Report and Review
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Case Report
Scleredema — Rare skin manifestation in diabetic
Hemant Thacker1, Janardan Nimbolkar1,*, Bhavesh Gandhi1
1Dept. of Critical Care Medicine, Breach Candy Hospital, Mumbai, Maharashtra, India
ARTICLE INFO
Article history:
Received 15-09-2021
Accepted 20-12-2021
Available online 08-02-2022
Keywords:
Diabetic
Scleredema
ABSTRACT
Scleredema is a connective tissue disorder of unknown etiology.
Case Report: A 44 years diabetic, obese female patient, admitted with edema feet, erythematous, indurated
hyper pigmentation with ascending progression to trunk which rapidly transformed to painless dark
pigmented blister and hard non-pitting edema of anterior abdominal wall. In a week time. She was managed
with broad spectrum antibiotics, was aggressively diuresed along with albumin infusion. Her skin biopsy
was done which revealed scleredema. She had poor LV function due to volume overload or cardiomyopathy.
After initial improvement on 14t h day, she suddenly developed fulminant septicemia. Within hours, she
displayed multi organ dysfunction and the heart and kidney which were already delicately balanced,
decompensated. There was intractable arrhythmias and severe LV dysfunction, renal failure and ischemic
hepatitis following severe hypotension.
Discussion: Scleredema in diabetic subjects, can be an ominous sign for producing fulminant multiorgan
dysfunction and can present as cardiomyopathy, myocarditis, liver dysfunction etc., and these patients are
more prone to infections or may be vice versa. Scleredema can be considered as a marker of fulminant
illness in immunocompromised individuals. There is a need for treatment protocols or research into this a
rather enigmatic but rare illness.
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1. Introduction
Scleredema Diabeticorum is an infrequent connective
tissue disorder characterized by thickening, hardening, and
painlessness of the affection of skin, was first described
by Buschke in the year 1900.1The term scleredema is a
misnomer because neither sclerosis nor edema is found on
microscopic examination. Sometimes, it just presents with
erythema and pigmentation of the skin without any clear
demarcation between involved and normal skin.
There are three clinical forms of scleredema, which are
classified by their associated condition are, associated with a
history of an antecedent infection (type 1), a blood dyscrasia
(type 2), or diabetes mellitus (type 3). Each of these clinical
* Corresponding author.
E-mail address:dr.janardannimbolkar@gmail.com (J. Nimbolkar).
forms has a different history, course, and prognosis.2Here
we are reporting a care of scleredema with multiorgan
involvement.
2. Case Report
This 44 years old female, obese, known case of type2
Diabetes mellitus usually well controlled with medication,
was in apparently alright with good effort tolerance till 3
months back, when is noticed swelling over both feet which
were pitting in nature initially and gradually progressed till
trunk and abdomen, since last few days rapid progression
was observed along with complains of distension of
abdomen with non-pitting edema. Feet and abdomen were
rock hard in consistency. Hyperpigmentation of skin started
in lower extremities more on anterior and medial aspect of
https://doi.org/10.18231/j.sajcrr.2021.005
2319-1090/© 2021 Innovative Publication, All rights reserved. 26

Thacker, Nimbolkar and Gandhi / Southeast Asian Journal of Case Report and Review 2021;8(1):26–29 27
Fig. 1: Leg indurated, edematous, non-pitting, blackening
Fig. 2: Thigh, edematous, non-pitting, blisters
Fig. 3: Abdomen distended, hard on palpation
Fig. 4: Histopathology A) Skin biopsy Hematoxylin & Eosin 20
X. Dermis is markedly expanded by thickened collagen bundles,
not accompanied by increase in number of fibroblasts. Stains for
Amyloid were negative. (B) Congo Red stain and C) Polarized
microscopy, no apple green birefringence
thighs since last 3 months but last 7 days it was looking
blacker and redder and progressed to abdomen. There were
blisters and vesicles (as seen in Figures 1, 2 and 3) hence
she got admitted to our hospital for further treatment.
Patient was extensively investigated. Her blood
biochemistry were, (ESR 18, SGOT- 29, SGPT- 25, S.
Bilirubin- 1.8,S.Albumin -3.08, WBC-18000, Hb- 13.6,
platelets- 366, BNP- 2600, Creatinine- 0.6.) suggestive of
slightly low albumin levels with leukocytosis with raised
BNP indicating fluid overload which was confirmed on The
2 D echo revealing Generalized LV hypokinesia, dilated
chamber, dilated IVC with reduced LVEF – 25 %. USG
abdomen revealed hepatomegaly with moderate ascites.
Serology for RA, ANA, Anti DNA, ASMA, ENA profile,
anti-mitochondrial antibody as part of connective tissue
disorder work up done but were negative. For non-pitting
edema work up for thyroid was in form blood level
T3, T4, TSH was done but turn out be normal. Serum
Electrophoresis showed raised band in Beta globulin region
with raised serum IgG -1657 level indicating possible
gammopathy, Bone marrow biopsy was done which ruled
out Multiple Myeloma and it showed a reactive marrow with
plasma cells 4 %. Even CT scan abdomen was done but
could not add more, except suggestive of large amount of
ascites and mild hepatomegaly.
Hence finally skin biopsy was attempted after consulting
dermatologist which was negative for Myeloma and
Amyloid but findings were consistent with Scleredema as
seen in Figure 4.

28 Thacker, Nimbolkar and Gandhi / Southeast Asian Journal of Case Report and Review 2021;8(1):26–29
3. Management
After through and extensive investigation, Clinical and
biopsy diagnosis of scleredema with LV dysfunction was
made and patient started on Aldactone and Torsemide
as well as ACE inhibitors. Patient was kept in negative
balance more than 1000ml/day almost every day. In view
of high WBC count and clinical picture patient was
treated with Daptomycin to cover for a cellulitis and was
deescalated and stop after 10 days. Patient was afebrile
for 14 days during hospitalization. As patient was showing
clinical improvement with passing good amount of urine
without diuretics and was mobilized as walking with
support as she was clinically stable. On 15day patient
developed very high-grade fever of 105 F, without chills
and rigors and became restless, progressively drowsy,
breathless with decrease in urine output, was shifted to
ICU for further management. In ICU X-ray chest showed
bilateral basal haziness. Patient had severe hypotension
required high doses of Noradrenaline, vasopressor supports.
Patient became Anuric, K+ 6.9, ABG suggests severe
acidosis, S. Bilirubin- 6.4, INR 6.4, Procalcitonin -3.5,
SGOT- 17000, SGPT- 1800, WBC- 30000, suggestive of
severe septic shock with multi-organ dysfunction. She was
started with IV Meropenem and Vancomycin as broad-
spectrum antibiotic cover and stress dose of steroid. Patient
was electively intubated in view of hypoxia, acidosis
and hemodynamic instability and put on ventilator. FFPs
transfusions were given in view of very high INR. Patient
was immediately dialyzed. ECG was initially sinus rhythm
later showing RBBB with cardiac enzymes and troponins
positive. She was getting multiple runs of ventricular
tachycardia requiring DC shocks in view of worsening
hemodynamics along with amiodarone. With dialysis K+
and acidosis was corrected but in spite of that patient was
getting arrhythmias, hence balloon tip pacemaker was put
and overdrive pacing was tried and with that incidence
of arrhythmias had reduced. Her inotropes requirement
was persistently increasing. Neurological deterioration was
very rapid that within 24 hours patient was comatose, as
there was no response to pain stimulus and deep tendon
reflexes were absent with mute planters, however her
pupils were reacting to light with ventilator trigger and
cough reflex were present. Next day there was further
worsening in neurological, clinical, hemodynamically as
well as laboratory parameters as WBC — 40000, Hb-
13, platelets 77, SGOT 32489, SGPT- 6676, Bilirubin-
7.55, PT more than 2 min. Blood culture, swab of
fluid from blisters and fluid culture showed no growth.
Patient’s conditions rapidly deteriorated with increased
inotropic requirement, intractable arrhythmias, and severe
derangement in coagulation as well as worsening liver
function test. In spite of aggressive management, she
succumbed to her illness within 72 hours of ICU admission.
4. Discussion
Scleredema is usually a clinical diagnosis because of its
typical skin manifestation but many a times requires skin
biopsy for Definite diagnosis as microscopic features of
the biopsy are characterized by thickening of the dermis
due to enlarged collagen bundles in deep reticular dermis
with clear spaces between them, filled often with mucin
deposits, which are inconstant and not necessary for the
diagnosis.3There is some strong link reported in association
with diabetes mellitus called scleredema diabeticorum,
with isolated reported cases of monoclonal gammopathy,
multiple myeloma in some patients of scleredema.
This unusual skin alignment, various treatment option
and modalities has been tried like immunosuppressive drug
such as systemic steroids, cyclosporine,4methotrexate, or
high-dose penicillin, or various modes of phototherapy,
such as UVA1, narrow-band UVB, psoralen with ultraviolet
light A (PUVA) either administered systemically or
topically,5penicillamine, electron beam, and glycemic
control with prostaglandin E1 (PGE1), have all been tried
with limited success. In myeloma related scleredema,
there are evidence of chemotherapy suppression for
myeloma resulted in concomitant improvement of the
skin disease and better prognosis.6For patients with
paraproteinemia, extracorporeal photopheresis has been
used, but with variable results. Recently, multiple reports
had described intravenous immunoglobulin (IVIg) therapy
especially in postinfectious, diabetic, and monoclonal
gammopathy–associated scleredema that were refractory to
more standard therapies with better efficacy.7
In our patient diabetes was one of the predisposing
risk factor. We have ruled out monoclonal gammopathy
and myeloma in our case as bone marrow biopsy was
suggestive of 4% plasma cells and reactive marrow. In our
patient the most important key factor was high WBC count
and LV dysfunction at time of admission. Hence, she was
treated with IV daptomycin (as broad spectrum) to cover
gram positive organism in view of suspicious cellulitis.
There was severe LV dysfunction so possibility of sepsis
related or associated myocarditis could not be ruled out.
There was liver dysfunction with mild hepatomegaly and
severe ascites. There was no feature of cirrhosis. Due to
severe sepsis and refractory shock, there was acute kidney
injury, with hyper kalmia and intractable arrhythmias and
required urgent dialysis. All blood cultures, fluid from
blisters and tissues from wound were negative for any
organisms. There was poor response in spite of prompt
treatment with antibiotics and steroids.8So, this was a case
of diabetes mellitus with sepsis and fulminant multiorgan
dysfunction and the presenting feature was scleredema
which unfortunately assumed a fulminant form. A review
of the literature reveals very limited treatment guidelines
especially of the non-fulminant form.

Thacker, Nimbolkar and Gandhi / Southeast Asian Journal of Case Report and Review 2021;8(1):26–29 29
5. Conclusion
Appropriate prophylactic antibiotic therapy should be
started in diabetes mellitus patients presenting with
scleredema, because it’s a warning sign of preceding
fulminant infection, although antibiotics do not appear to
shorten the course of skin findings in scleredema.9The role
of steroids or immunosuppressants in fulminant conditions
associated with sepsis still needs to be determined. Our case
brought out the high mortality associated with Scleredema
with comorbidities for which much need for further protocol
and studies.
6. Source of Funding
None.
7. Conflict of Interest
None.
References
1. Buschke A. Uber scleroedema. Klin Wochenschr; 1902. p. 39955–7.
2. Rho YW, Suhr KB, Lee JH, Park JK. A clinical observation of
scleredema adultorum and its relationship to diabetes. J Dermatol.
1998;25(2):103–7.
3. Rongioletti F, Rebora A. Cutaneous mucinoses: microscopic
criteria for diagnosis. Am J Dermatopathol. 2001;23(3):257–67.
doi:10.1097/00000372-200106000-00022.
4. Sommer LL, Heymann WR. Fulminans in dermatology: a call to action:
a recommendation for consideration of the term scleredema fulminans.
J Clin Aesthet Dermatol. 2014;7(6):42–5.
5. Yoshimura J, Asano Y, Takahashi T, Uwajima Y, Kagami S, Honda
H. A case of scleredema adultorum successfully treated with narrow-
band ultraviolet B phototherapy. Mod Rheumatol. 2014;26(2):302–6.
doi:10.3109/14397595.2013.875640.
6. Bray SM, Varghese S, English JC. Ultrasonic massage and physical
therapy for scleredema: improving activities of daily living. Arch
Dermatol. 2010;146(4):453–7. doi:10.1001/archdermatol.2010.31.
7. Barde C, Masouyé I, Saurat JH, Gal L, A F. [Scleroedema adultorum
Buschke in a diabetic subject: intravenous immunoglobulin
therapy]. Ann Dermatol Venereol. 2009;136(4):360–3.
doi:10.1016/j.annder.2008.09.024.
8. Sommer LL, Heymann WR. Fulminans in dermatology: a call to action:
a recommendation for consideration of the term scleredema fulminans.
J Clin Aesthet Dermatol. 2014;7(6):42–7.
9. Rani JD, Patil SG, Murthy ST, Koshy AV, Nagpal D, Gupta S. Juvenile
scleredema of Buschke. J Contemp Dent Pract. 2012;13(1):111–5.
Author biography
Hemant Thacker, Consultant Physician
Janardan Nimbolkar, Intensivist
Bhavesh Gandhi, Intensivist
Cite this article: Thacker H, Nimbolkar J, Gandhi B. Scleredema —
Rare skin manifestation in diabetic. Southeast Asian J Case Rep Rev
2021;8(1):26-29.