Tsutomu recently evaluated the effectiveness of cyclosporine
treatment in chronic idiopathic urticaria (CIU). Some studies
showed that high CIU activity can be associated with an elevated
high sensitivity C-reactive protein (HS-CRP) and a decreased
basophil counts. Patients with CIU and elevated CRP levels had
a significant decrease in CRP levels as the urticaria improved
with cyclosporine treatment.51
Kessel and Toubi also reported, good results for chronic urticaria
with low-dose cyclosporine (3/mg/kg/day). They used
this dose for 2 months and then the dose was gradually decreased
to 2 mg/kg/day and 1 mg/kg/day during the following
month and concluded that it is an effective and safe therapy
regimen. They also suggested that in a small subgroup of
patients, long-term therapy could be necessary, and it is considered
relatively safe.52
In this study the rate of infections with Epstein Barr virus and cytomegalovirus
was the same as would be expected in a normal
population. Also the rates for malignancy would be expected to
be low as long as the levels were kept at or below 50 ng per mL.
They concluded that when cyclosporine is required for longer
periods (5 to 10 years) for the treatment of CIU, the goal should
be to keep the dose between 1 mg per kg to 1.5 mg per kg.51
Prurigo Nodularis
Prurigo nodularis is an idiopathic condition consisting of nodular cutaneous lesions that itch intensely. It is a reaction associated with a marked proliferation of sensory nerve fibers associated with severe itch. Although some acute forms may be induced by insect stings, most of the subacute and chronic forms appear to be idiopathic. Several metabolic, psiquiatric, infectious, and malignant disorders may be associated with prurigo nodularis lesions.1,53
Prurigo nodularis is an idiopathic condition consisting of nodular cutaneous lesions that itch intensely. It is a reaction associated with a marked proliferation of sensory nerve fibers associated with severe itch. Although some acute forms may be induced by insect stings, most of the subacute and chronic forms appear to be idiopathic. Several metabolic, psiquiatric, infectious, and malignant disorders may be associated with prurigo nodularis lesions.1,53
The treatment for prurigo nodularis is extremely unsatisfactory.
Currently, treatments include topical antipruritics, topical
steroids, intralesional steroids, psoralen plus Ultraviolet A
light phototherapy, ultraviolet B light therapy, cryotherapy,
azathioprine, chloroquine, dapsone, minocycline, thalidomide,
topical vitamin D, and capsaicin.1,53
Cyclosporine may be considered a second line agent for this
condition. Frequently, high dosages of 3 mg/kg/day to 4.5 mg/
kg/day for 24 to 36 weeks are required. In some patients, significant
improvement in the lesion and reduction of pruritus may
be seen. Pruritus can be reduced as early as the first 2 weeks
of therapy, allowing also the prurigo nodules to heal. In most
reports showing that when cyclosporine dosage is reduced or
discontinued the prurigo relapses.53,54
The mechanism of action of cyclosporine in nodular prurigo is
believed to be similar to its action in atopic dermatitis.53,55
Neutrophilic Dermatosis
Pyoderma Gangrenosum
The use of cyclosporine as a second-line therapy in corticosteroid refractory pyoderma gangrenosum (PG), or as coadjuvant treatment, has been well documented with multiple cases reported in the last 2 decades. The immunomodulator and antinflammatory effects achieved with cyclosporine have proven to be effective in the management of this condition. Occasionally cyclosporine maybe of beneficial in secondary associated systemic diseases such as inflammatory bowel disease.1,56,57
Pyoderma Gangrenosum
The use of cyclosporine as a second-line therapy in corticosteroid refractory pyoderma gangrenosum (PG), or as coadjuvant treatment, has been well documented with multiple cases reported in the last 2 decades. The immunomodulator and antinflammatory effects achieved with cyclosporine have proven to be effective in the management of this condition. Occasionally cyclosporine maybe of beneficial in secondary associated systemic diseases such as inflammatory bowel disease.1,56,57
Although there is no standard protocol for the treatment of PG
with cyclosporine, the majority of cases of success reported in
the literature were dosed with 5 mg/kg/day.1,58,59
It has been suggested that smaller doses may not be as effective.
Reich et al58 and Soria et al60 reported loss of efficacy as well as
the development of new lesions, when oral doses were reduced
from 5 mg/kg/day to 3 mg/kg/day. Nonetheless, some of their
patients benefited equally from a lower dose (3 mg/kg/day).
Oral administration of cyclosporine was the most commonly
prescribed, however, other authors have noted that giving cyclosporine
IV at a dose drug of 3 mg/kg/day for 1 week,61 or at 4
mg/kg/day for 1 to 3 weeks produce similar results.62
Formulations, other than systemic, have also been used for the
treatment of PG. Azizan et al63 treated 4 patients with topical cyclosporine
with significant efficacy and without significant systemic
absorption of the drug and no side effects. This may be an ideal
therapeutic approach for localized disease and/or for PG cases that
are not associated systemic conditions. This option may represent
a safer alternative for very ill patients, thus preventing the potential
of side effects associated with the systemic use of this drug.63-65
Once therapy is implemented, improvement of the inflammation
surrounding the ulcer may be evident within 24 hours after
starting treatment with peak improvement noticeable after
2 weeks. Measuring the efficacy of treatment is challenging,
however, as these patients are usually concomitantly receiving
other immunosuppressant drugs.
The incidence of relapse is very high despite initial improvement;
nevertheless, cyclosporine may represent a more affordable option
to treat PG in cases were treatment with other options such as
biologics (infliximab, adalimumab) is not economically feasible.
Sweet's Syndrome
Sweet's syndrome, pyoderma gangrenosum, and subcorneal pustular dermatosis are neutrophilic conditions that have an inflammatory infiltrate consisting of mature polymorphonuclear leukocytes. The neutrophils are usually located within the dermis
Sweet's syndrome, pyoderma gangrenosum, and subcorneal pustular dermatosis are neutrophilic conditions that have an inflammatory infiltrate consisting of mature polymorphonuclear leukocytes. The neutrophils are usually located within the dermis
