Observation |

Neutrophilic Lobular Panniculitis as an Expression of a Widened Spectrum of Familial Mediterranean Fever

María Leiva-Salinas, MD1; Isabel Betlloch, MD, PhD1; María Paloma Arribas, MD1; Laura Francés, MD1; Jose Carlos Pascual, MD1
[+] Author Affiliations
1Department of Dermatology, Hospital General Universitario de Alicante, Alicante, Spain
JAMA Dermatol. 2014;150(2):213-214. doi:10.1001/jamadermatol.2013.6095.
Text Size: A A A
Published online


Familial Mediterranean fever (FMF) is considered to be an autosomal recessive disease, though it is controversial.13 The marenostrin-encoding fever gene (MEFV) is responsible for FMF. The most frequent mutation is M694V, which represents a genetic risk factor for development of amyloidosis1 and more severe disease. The classic clinical picture consists of generally short recurrent febrile episodes, serositis, and erysipelas-like erythema (ELE). Colchicine is the treatment of choice for prevention of the attacks and AA amyloidosis. With isolation of the MEFV gene in 1997, incomplete or “atypical” forms of FMF have been reported and are considered by some authors an expanded spectrum of the disease.2

Figures in this Article

Sign In to Access Full Content

Don't have Access?

Register and get free email Table of Contents alerts, saved searches, PowerPoint downloads, CME quizzes, and more

Subscribe for full-text access to content from 1998 forward and a host of useful features

Activate your current subscription (AMA members and current subscribers)

Purchase Online Access to this article for 24 hours

First Page Preview

View Large
First page PDF preview


Place holder to copy figure label and caption
Figure 1.
Recurrent Contusiform Nodules on the Lower Limbs

Tender, erythematous, contusiform, warm nodules with irregular shape, located on the lower limbs.

Graphic Jump Location
Place holder to copy figure label and caption
Figure 2.
Lobular Neutrophilic Panniculitis

Punch biopsy specimens were taken and stained with hematoxylin-eosin. A, Predominantly lobular neutrophilic panniculitis without evidence of necrosis, vasculitis, crystals, or destruction of adipocytes (original magnification ×20). B, Lipophagic histiocytes and giant cells (original magnification ×40).

Graphic Jump Location




Meets CME requirements for:
Browse CME for all U.S. States
Accreditation Information
The American Medical Association is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians. The AMA designates this journal-based CME activity for a maximum of 1 AMA PRA Category 1 CreditTM per course. Physicians should claim only the credit commensurate with the extent of their participation in the activity. Physicians who complete the CME course and score at least 80% correct on the quiz are eligible for AMA PRA Category 1 CreditTM.
Note: You must get at least of the answers correct to pass this quiz.
You have not filled in all the answers to complete this quiz
The following questions were not answered:
Sorry, you have unsuccessfully completed this CME quiz with a score of
The following questions were not answered correctly:
Commitment to Change (optional):
Indicate what change(s) you will implement in your practice, if any, based on this CME course.
Your quiz results:
The filled radio buttons indicate your responses. The preferred responses are highlighted
For CME Course: A Proposed Model for Initial Assessment and Management of Acute Heart Failure Syndromes
Indicate what changes(s) you will implement in your practice, if any, based on this CME course.
Citing articles are presented as examples only. In non-demo SCM6 implementation, integration with CrossRef’s "Cited By" API will populate this tab (http://www.crossref.org/citedby.html).
Submit a Comment


Some tools below are only available to our subscribers or users with an online account.

Sign In to Access Full Content

Related Content

Customize your page view by dragging & repositioning the boxes below.

Articles Related By Topic
Related Topics
PubMed Articles