Page 102 - The Netter Collection of Medical Illustrations - Integumentary System_ Volume 4 ( PDFDrive )

Plate 4-17 Integumentary System

CUTANEOUS MANIFESTATIONS OF LUPUS

CUTANEOUS LUPUS (Continued)

Treatment includes keeping the regions dry and warm
by avoiding cold exposure. Patients diagnosed with
pernio probably should undergo screening for lupus,
because a small percentage of them actually have lupus
chilblains. Histological evaluation of lupus chilblains
shows a dense lymphocytic infiltrate with some areas
of thrombosis of small vessels and a lymphocytic
vasculitis.
The cutaneous findings seen in SLE are vast and can
overlap with other forms of cutaneous lupus. Although
the systemic findings are responsible for the morbidity
and mortality, the cutaneous findings are often the pre-
senting sign, and, if the clinician is aware, they can help Neonatal lupus. Neonatal lupus is transient
make the diagnosis. The most important of the cutane- in nature and is caused by maternal anti-
bodies that cross the placenta. Newborns
ous skin findings in SLE is the malar rash. This rash are at risk for developing heart block.
manifests as a tender, pink-to-red plaque or patch on The cutaneous findings eventually resolve
the cheeks and nose, mimicking the shape of a butterfly; spontaneously.
hence, it has been termed the “butterfly rash of lupus.”
It is commonly mistaken for rosacea, and vice versa.
Rosacea typically affects a wider area of skin and is
associated with more telangiectases and papulopustular
lesions. The malar rash of lupus also spares the nasola-
bial fold, which is an important clinical finding and a
discriminating objective discovery. It is typically more
prominent during systemic flares of the underlying
SLE, and patients can appear very ill. Patients are
exquisitely photosensitive, and the rash is exacerbated
by exposure to ultraviolet light.
Discoid lupus is also seen as a manifestation of sys-
temic lupus, and it has the same clinical appearance as
described earlier. Raynaud’s phenomenon is well
described, and a high percentage of patients with SLE
report those symptoms. Alopecia was long used to help
make the diagnosis of lupus. It is no longer part of the
diagnostic criteria, but it can have significant psycho-
logical impact on the patient. Nail and capillary nail
fold changes are seen if looked for. The true incidence
of these findings is unknown. Nail fold telangiectases
and erythema are the two most common nail findings.
Nail pitting, ridging, and alterations in the color of the
lunula have also been reported. Lupus patients with nail
changes have been found to have a higher incidence of
mucosal ulcerations, which are another of the mucocu-
taneous findings of SLE. Livedo reticularis is a fishnet-
like pattern found typically on the lower extremities; it
is a nonspecific finding but has been reported com-
monly in lupus. It also occurs in many other skin and
systemic diseases. Lupus chilblains. Tender red to purple macules
Histology: The histological findings in all forms of and papules on the feet. Exacerbated by cold
lupus are similar, with specific forms having some Lupus erythematosus disseminatus and wet environments
unique findings. Most forms show an interface derma-
titis with hydropic changes in the basilar layer of the
epidermis. A superficial and deep periadnexal lympho-
cytic infiltrate is almost universally seen. Other connec- Treatment: The treatment of cutaneous lupus is dif- immediately, and patients should be screened routinely
tive tissue diseases (e.g., dermatomyositis) can have ficult and must be tailored to the patient and the specific by their family physician or rheumatologist for progres-
similar histological findings. Discoid lupus may show form of lupus. Potent topical corticosteroids may work sion of the disease.
scarring, atrophy, and follicular plugging along with for a tiny lesion of discoid lupus, but they are not effec- Specific therapies for cutaneous lupus include oral
these other findings. Lupus panniculitis is unique in tive in lupus panniculitis. Universal treatment of cuta- prednisone and hydroxychloroquine or chloroquine as
that the inflammation is localized to the subcutaneous neous lupus requires sun protection and sunscreen use. the typical first-line agents. If these are unsuccessful,
tissue. The diagnosis of lupus panniculitis is difficult The sunscreen used should block in the UVA range, quinacrine can be added. Other agents that have been
and requires a host of special stains and clinical patho- because this is the most active form of ultraviolet light reported to be effective include dapsone, isotretinoin,
logical correlation. that exacerbates lupus. Smoking should be ceased and methotrexate.

88 THE NETTER COLLECTION OF MEDICAL ILLUSTRATIONS