The First Case of Rowell Syndrome with Lupus Nephritis and Lobar Pneumonia in a Male Child Reported in Pakistan.

Sumreen Shahid,Laila Tul Qadar,Maria Khan,Ammarah Jamal,Manahil Akmal

doi:10.7759/cureus.4604

Sumreen Shahid, Laila Tul Qadar + Show 3 more

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https://doi.org/10.7759/cureus.4604

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Abstract

Rowell syndrome (RS) is a rare entity that presents with lupus erythematosus (LE), erythema multiforme (EM) like lesions and characteristic immunological findings including positive rheumatoid factor (RF), speckled antinuclear antibody (ANA) and positive anti-Ro or anti-La antibodies in the serum. Only a few cases have been reported in the literature, mostly in middle-aged women. Our case is about an 11-year-old male child who had a history of pustular rashes over the whole body with scattered targetoid lesions for the past few months and later developed respiratory distress and swelling of the body. He was extensively investigated and confirmed on specific positive immunochemistry markers as RS along with lobar pneumonia (LP) and lupus nephritis. The co-existence of lobar pneumonia in our patient was a unique feature that has not been previously reported.

Highlights

Rowell syndrome (RS), first described in 1922 by Scholtz et al, was later characterized as a separate syndrome by Rowell and his colleagues in 1963 when they encountered four women who were suffering from systemic lupus erythematosus (SLE) and had developed rashes suggestive of erythema multiforme (EM) [1,2].Received 04/29/2019 Review began 05/02/2019 Review ended 05/02/2019 Published 05/07/2019Since 33 cases of RS have been reported in the literature, mostly affecting middle-aged women [3]
Rowell syndrome (RS) is a rare entity that presents with lupus erythematosus (LE), erythema multiforme (EM) like lesions and characteristic immunological findings including positive rheumatoid factor (RF), speckled antinuclear antibody (ANA) and positive anti-Ro or anti-La antibodies in the serum
In 2008, Zeitouni et al defined the major diagnostic criteria of RS consisting of preexisting lupus erythematosus (LE) getting superimposed by EM and speckled pattern of antinuclear antibody (ANA), along with at least one minor criterion consisting of positive rheumatoid factor (RF), chilblains or the presence of anti-Ro or anti-La antibodies [4]

Summary

Introduction

Rowell syndrome (RS), first described in 1922 by Scholtz et al, was later characterized as a separate syndrome by Rowell and his colleagues in 1963 when they encountered four women who were suffering from systemic lupus erythematosus (SLE) and had developed rashes suggestive of erythema multiforme (EM) [1,2]. Examination of the chest revealed signs consistent with left-sided LP He had a scaly rash with occasional targetoid lesions all over the body associated with peeling of the skin (Figure 1), ruptured blisters, loss of fingernails (Figure 2) and sparse, brittle and depigmented hairs. He had hepatomegaly with a liver span of 15 cm and signs of free fluid in the abdomen. The patient was treated with methylprednisolone initially followed by oral prednisolone, azathioprine, naproxen and hydroxychloroquine on the advice of pediatric rheumatologist and antibiotics for pulmonary infection His rashes improved and proteinuria resolved over a period of two weeks. The patient is under regular follow-up with no recurrence of skin lesions or pneumonia

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