The Neutrophilic Dermatoses

David Farhi, MD; Daniel Wallach, MD

Disclosures

Dermatology Nursing. 2008;20(4):274-282. 

In This Article

Extracutaneous Signs in Neutrophilic Diseases

Extracutaneous signs may be present in all ND, though mainly in SS and PG. Some of these extracutaneous signs are nonspecific and others are related to frequently associated autonomous diseases.

The most common nonspecific signs are fever, malaise, and diffuse pain. They may be present in all ND, and are part of typical SS.

Nonspecific joint pain (either axial or peripheral) that parallels the evolution of the cutaneous disease is also frequent. Less commonly, sterile neutrophilic arthritis may be present. These associated articular signs must be differentiated from arthritis occurring in the context of an associated chronic inflammatory joint disease.

Some manifestations may reflect the presence of deep organ invasion by neutrophils. Cough, dyspnea, and thoracic pain may reveal an associated neutrophilic lung disease, whose course parallels that of the neutrophilic dermatosis. Likewise, other extracutaneous neutrophilic involvements may be retrieved in various anatomical sites: pleural effusion; kidney dysfunction; recurrent chronic multifocal osteomyelitis; central nervous system involvement; cardiac, muscular, ocular involvement; and aseptic abscess of a deep organ (liver, spleen, lymph nodes, pancreas, etc).

This occurrence of a systemic multi-organ aseptic neutrophilic in filtration has been coined "the neutrophilic disease" (Wallach & Vignon-Pennamen, 2006). However, it should be emphasized that these deep organ manifestations remain infrequent.

Beside the extracutaneous neutrophilic manifestations described previously, some autonomous diseases, which may occur in the absence of any context of neutrophilic dermatoses, may be associated with ND, with a higher frequency that would be expected by chance.

As already mentioned, most if not all of these associated systemic diseases may accompany any of the ND. Hence, in terms of nosology, this is a further argument for the classification of all the ND into a single group of diseases.

Systemic diseases associated with neutrophilic dermatoses are summarized in Table 2 . They can be classified in three groups: blood disorders, digestive diseases, and chronic rheumatologic conditions. Other associated systemic diseases are rare.

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