scholarly journals Sneddon-Wilkinson Disease and Monoclonal Gammopathy of Undetermined Significance in the Elderly: Case Report

2019 ◽  
Vol 11 (2) ◽  
pp. 209-214
Author(s):  
Gabriele Ceccarelli ◽  
Elisa Molinelli ◽  
Anna Campanati ◽  
Gaia Goteri ◽  
Annamaria Offidani

Sneddon-Wilkinson disease (SWD) or subcorneal pustular dermatosis is considered a rare pustular skin disease with chronic relapsing course. An association between SWD and other chronic conditions, such as IgA or IgG monoclonal gammopathy of undetermined significance (MGUS), IgA myeloma, pyoderma gangrenosum, thyroid gland disorders, and neoplastic diseases other than MGUS/myeloma, is known. We describe the case of a 92-year-old male patient with SWD and a concurrent IgG MGUS who had been treated with systemic betamethasone, topical mometasone furoate, and methylprednisolone aceponate, with a complete and durable resolution of symptoms and skin lesions without side effects. Systemic and topical steroids were very effective and well tolerated in our patient. This is the second case reported in the literature on the efficacy of a corticosteroid regimen in SWD in a fragile patient. This therapeutic approach (instead of dapsone therapy) has been used due to its relatively good safety profile.

2015 ◽  
Vol 54 (12) ◽  
pp. e551-e553 ◽  
Author(s):  
Mukaddes Kavala ◽  
Ayse S. Karadag ◽  
Ilkin Zindancı ◽  
Zafer Turkoglu ◽  
Erman Ozturk ◽  
...  

2015 ◽  
Vol 10 (1) ◽  
pp. 71
Author(s):  
Ozturk Ates ◽  
Kemal Kosemehmetoglu ◽  
Gunes Guner ◽  
Yusuf Bayraktar

A 69-year-old man noticed abdominal pain located on right upper quadrant. Physical examination showed hepatosplenomegaly and icteric discoloration of sclera. On evaluation, patient was diagnosed to have hepatic amyloidosis related monoclonal gammopathy of undetermined significance (MGUS) and sinusoidal obstruction syndrome with intrahepatic cholestasis. In this case report we emphasize fulminant hepatic failure due to primer amyloidosis in diagnosed with MGUS patient.


2017 ◽  
Vol 9 (3) ◽  
pp. 124-128
Author(s):  
Svetlana Popadić ◽  
Mirjana Gajić-Veljić ◽  
Biljana Marenović ◽  
Miloš Nikolić

Abstract Mastocytosis refers to a group of diseases characterized by a clonal proliferation and accumulation of mast cells in one or more tissues/organs with different clinical presentations. In children, limited cutaneous forms of mastocytosis are rather frequent, while systemic mastocytosis is rare. The diagnosis of cutaneous mastocytosis is based on clinical findings and histopathology. We present a patient who developed skin lesions at the age of 18 months. Clinical findings, confirmed by histopathology, were consistent with diffuse cutaneous mastocytosis. The follow-up period was 7 years. The treatment included oral antihistamines in combination with mast cell stabilizers, mild topical steroids and avoidance of friction. During the follow-up period, there were no signs of systemic involvement, and the quality of life was preserved, despite the large surface of affected skin. This case report should increase the awareness and knowledge of clinicians about this rare form of cutaneous mastocytosis in the pediatric population.


2020 ◽  
Vol 20 ◽  
pp. S291
Author(s):  
Ahmad Khalil ◽  
Dany AbiGerges ◽  
Pamela Sfeir ◽  
Edmond Abboud ◽  
Hussein Farhat ◽  
...  

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