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Journal of the American Academy of Dermatology
Volume 48, n° 2S
pages 11-14 (février 2003)
Doi : 10.1067/mjd.2003.126
Multicentric reticulohistiocytosis presenting with clinical features of dermatomyositis

Sherry H. Hsiung, Edward F. Chan, MD, Rosalie Elenitsas, MD, Sharon L. Kolasinski, MD, H.Ralph Schumacher, MD, Victoria P. Werth, MD
Philadelphia, Pennsylvania 
From the Department of Dermatologya and Division of Rheumatology,b University of Pennsylvania School of Medicine 


Multicentric reticulohistiocytosis (MRH) is a rare disorder with proliferating histiocytes that develop into multinucleated giant cells with “ground-glass” cytoplasm. The disease presents with a rapidly destructive, sometimes permanently debilitating, polyarthritis and a papulonodular eruption, generally of the face and hands. We present 3 cases of MRH in which the initial clinical diagnosis was thought to be dermatomyositis (DM). The cutaneous findings in these cases included an erythematous, predominantly photodistributed macular and papular eruption clinically consistent with DM. However, skin biopsy specimens revealed the diagnosis of MRH. This previously unreported clinical similarity between MRH and DM is significant because the treatment options and future complications vary greatly between the 2 diseases. Thus, it is important to consider the possibility of multicentric reticulohistiocytosis when evaluating a patient with the clinical diagnosis of dermatomyositis. (J Am Acad Dermatol 2003;48:S11-4.)

The full text of this article is available in PDF format.

 This supplement is made possible through an unrestricted educational grant from Stiefel Laboratories to the American Academy of Dermatology.
 There were no sources of funding, whether commercial or institutional, in the writing of this article.
 Reprint requests: Victoria P. Werth, MD, Second Floor Rhoads Pavilion, University of Pennsylvania Medical Center, 3400 Spruce St, Philadelphia, PA 19104. E-mail:
 0190-9622/2003/$30.00 + 0

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