Transcript ***** 1 - Dermatology Conferences

CUTANEOUS SARCOIDOSIS
Professor Svyatenko T.V.
Dnepropetrovsk Medical Academy, Ukraine
Center Dermatology and Cosmetology,
Dnepropetrovsk, Ukraine
Introductions
• Sarcoidosis is a multisystem disease that may involve
almost any organ system; therefore, it results in various
clinical manifestations.
• Cutaneous sarcoidosis occurs in up to one third of
patients with systemic sarcoidosis.
• Recognition of cutaneous lesions is important because
they provide a visible clue to the diagnosis and are an
easily accessible source of tissue for histologic
examination.
• Because lesions can exhibit many different
morphologies, cutaneous sarcoidosis is known as one of
the “great imitators” in dermatology. Specific
manifestations include papules, plaques, lupus pernio,
scar sarcoidosis, and rare morphologies such as
alopecia, ulcers, hypopigmented patches, and
ichthyosis.
• Most authors divide lesions of cutaneous sarcoidosis
into nonspecific and specific types. Although
nonspecific lesions occur in association with systemic
sarcoidosis, no granulomas are found on biopsy.
Specific lesions display noncaseating granulomas on
biopsy
• Correctly diagnosing sarcoidosis may be a challenge.
Unfortunately, no single test can prove the diagnosis.
Patients are diagnosed with sarcoidosis when a
compatible clinical or radiologic picture is present,
along with histologic evidence of noncaseating
granulomas, and when other potential causes, such as
infections, are excluded
• The treatment of cutaneous sarcoidosis is often frustrating, because
lesions may be refractory to treatment or may recur following
successful treatment. For localized involvement, topical or intralesional
steroids are used. Physicians frequently attempt to use superpotent
topical steroids because of their occasional effectiveness. However,
these steroids often do not adequately penetrate the skin lesion.
Intralesional steroids (e.g., triamcinolone acetonide [Kenalog] in a
dosage of 5 mg per mL) are typically more effective, with injections
repeated at two- to three-week intervals.
• Systemic agents are reserved for widespread, progressive lesions or
those that impair function. Systemic glucocorticoids are the most
effective agents. They are commonly used at slow, tapering dosages,
starting at 20 to 40 mg of oral prednisone daily for four to six
weeks. However, there are many drawbacks to this therapy
• Many other medications may be used in refractory cases, including
such agents as hydroxychloroquine (Plaquenil), methotrexate
(Rheumatrex), and thalidomide (Thalomid).
• Although randomized, controlled trials are lacking, multiple anecdotal
reports suggest the efficacy of these agents.
RAJANI KATTA Cutaneous Sarcoidosis: A Dermatologic Masquerader //
Am Fam Physician. 2002 ,15;65(8):15811585.
CASE № 1. CUTANEOUS SARCOIDOSIS
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49-year-old male patient, Caucasian
Reason for visit skin rash in the area of the upper third of the back slightly itching after
exercise in the summer time.
Medical history: according to the patient, the rash appeared all of a sudden 2 years
ago, with no pain or severe itching. No triggering accidents can be recalled.
The process progresses slowly. He repeatedly contacted dermatologists and was
treated with the diagnosis: multiple warts? Lichen planus? Atopic dermatitis? – without
any effects, also after administration of the local and oral corticosteroids therapy a
positive effect was observed for a short period of time.
On the medical examination no physical complaints were made. Patient also has a
history of atopic dermatitis when was a child, androgenetic hair loss. The parents and
close relatives are healthy. Job is not associated with negative factors.
During the examination patient physical condition was satisfactory.
Pathological skin process is localized on the upper back. Rush is represented by single
knots, up to 2 - 5 mm in diameter, multiple scattered and confluent lesions up to 3 cm
in diameter, fused into plaques, the colour red and pink, pink stagnant, with
telangiectasias, soft-elastic consistency, the surface smooth and shiny. On diascopy
symptom of "dust", the phenomenon of "cape".
Blood test results: normal range
Punch biopsy was provided
Histological investigation
showed epidermis without
changes, multiple epithelioid
- cell granulomas are
determined in papillary and
reticular dermis with the
presence of single giant
polynuclear cells.
Granulomas have clear
boundaries, with the
presence of single
lymphocytes around some of
them. When painting on
Alcian blue PAS + cells of
fungi have been identified.
The basement membrane is
within norm. No dermal
mucin.
• Sarcoidosis is one of the most common
granulomatosis. While there is a steady gradual
increase in the frequency of this disease. An
important feature is the presence of nosology
many similarities with tuberculosis,
lymphoproliferative and other cancers , various
autoimmune diseases. In sarcoidosis often affects
many organs, in this regard, the ill person may
apply to the primary physicians of different
specialties: dermatologist, ophthalmologist,
neurologist, surgeon, oncologist.
CASE № 2. SARCOIDOSIS COMBINED WITH
SKIN LESIONS
• 46-year-old male patient,
Caucasian
• Reason for visit:
- skin lesions since age 34
• Medical history:
– No personal history of any skin
diseases previously, otherwise in
good general health.
– For the last 34 years from the
first manifestation of skin
lesions, the patient consulted
various doctors
– Patient was treated for lichen
planus, lichen simplex chronicus
Vidal, psoriasis
– Laboratory findings were within
normal range
– Skin biopsy was performed.
Histological
investigation showed
productive chronic
inflammation with the
formation of epithelioid
cell granulomas, the
presence of
multinucleated giant
cells Pirogov-Langhans.
Information of common status
Spiral computed tomography of the thoracic
cavity : multiple foci of 1-2 mm d in both
lungs, increased pulmonary pattern, the
expansion of the lung roots mediastinal
lymphadenopathy. According to ultrosound,
peripheral lymph nodes: generalized
hyperplasia of the peripheral lymph nodes,
splenomegaly.
CASE № 2. SARCOIDOSIS COMBINED
WITH SKIN LESIONS
• 76-year-old male patient, Caucasian
• Reason for visit:
- skin lesions since age 72
• Medical history:
– No personal history of any skin diseases previously,
otherwise in good general health.
– For the last 72 years from the first manifestation of
skin lesions, the patient consulted various doctors
– Patient was treated for lichen planus? lymphoma?
– Laboratory findings were within normal range
– Skin biopsy was performed.
Information of common status
• Spiral computed tomography of the thoracic
cavity : multiple foci of 3-4 mm d in both lungs,
increased pulmonary pattern, the expansion of
the lung roots mediastinal lymphadenopathy.
Spiral computed tomography of the lumbar spine
spondyloarthrosis with disc protrusion, diffuse
osteoporosis
• According to ultrosound, peripheral lymph
nodes: generalized hyperplasia of the peripheral
lymph nodes, splenomegaly. According to
ultrosound - prostatitis
• Histological
investigation showed
productive chronic
inflammation with the
formation of epithelioid
cell granulomas, the
presence of
multinucleated giant
cells Pirogov-Langhans.
• Results immunohistochemistry
- granulomatous
inflammation with the
presence of giant cells
of Pirogov-Langhans,
sarcoidosis
CONCLUSION
• 1. In our opinion, the case described introduce
certain interest in the daily practice of a physician
- clinician.
• 2. This condition is rare and difficult to diagnose.
In this case, the punch - biopsy followed by
histological examination of biopsy is an important
diagnostic tool to verify the diagnosis of skin
sarcoidosis.
• 3. With correct diagnosis and proper
management, most patients with sarcoidosis
continue to lead a normal life.
Thank you for attention!