1. A Case Of Bad Itch
Dr . Zareen Mohamed
Consultant Allergist
Mehta Hospitals
2. Patient Presentation
• A 45 year gentleman presented to our allergy
clinic with symptoms of hives and intense pruritis
for the past 3 months.
• He complained that the rash and itching is more
prominent in the night .He complains of
considerable distress from the rash which he
describes it as of burning quality
• He also complains of frequent thirst and dryness
of skin despite adequate fluid intake
3. Patient presentation
• The hives are present for the past three to
four months and are partially controlled
with certizine 10 mg q.h.s.
• No history of exposure to new medication,
dietary supplements, naturopathic
medicines, cosmetics or recent travel
• No history of any significant viral or
bacterial infection prior to the onset of
hives
5. Past Medical History
• The patient did not complain of similar
episodes in the past
• No significant past medical or surgical
history
• No family history of Urticaria
• Not a Hypertensive or diabetic
• Family history insignificant for autoimmune
disease
7. Investigations
CBC- Normal
Hb/TC/DC/ESR- Normal
Liver function test/ Renal function test- Normal
Normal urine analysis
Food Allergy panel for serum specific
antibodies by allergy prick test - Normal
Normal Thyroid function with no anti thyroid
antibodies
8. Patient Presentation
• The patient was advised to change to non sedating antihistamines
and advised to follow up in 3 weeks for review
• The patient visited the clinic after 2 weeks with increasing pruritus
and decreasing exercise tolerance
• O/E: No hives, dryness of skin noted
Non tender firm lymph nodes noted in the cervical region
• He gave a similar history of lymph node swelling one year back
when he underwent FNAC of his lymph nodes , and was treated
symptomatically for Kikuchi fujimoto diease
9. Investigations
• Peripheral smear – Normal
• Serum calcium – 9.2 mg/dl
• Antinuclear antibodies – Negative at 1: 40
dilution
• CXR – Lungs - small calcific foci at bilateral
apex. Possibly healed granuloma.
• Serum LDH- Normal
• Serum Uric acid – 5.4 mg/dl
• Ultrasound of the neck
10. Investigations
USG NECK
• Thyroid –no nodes/cyst/calcification
• 3 or 4 enlarged lymph nodes seen in Rt side of
neck largest measures 2.6 * 0.6 cm, multiple
enlarged , edematous ,oval or spherical shaped
lymph nodes is seen in left side of neck and
supraclavicular region, largest measures 2.9*
1.5 cm.
11. USG NECK
• Few nodes show small cystic necrosis
within lymph nodes. Few nodes show
punctata echogenic focus within lymphatic
node with calcification or caseation
necrosis with no increased vascularity
within it
Imp: Bilateral cervical lymphadentis -
suggested fnac/hpe correlation.
12. Investigations
CXR LUNG
• small calcific foci at bilateral apex.
Possibly healed granuloma.
• TISSUE BIOPSY/PET SCAN/ IHC – all
suggestive of Hodgkin’s Lymphoma stage
11 a
13. Hodgkin’s lymphoma
• - Hodgkin lymphoma (formerly called
Hodgkin's disease) is a group of cancers
characterized by Reed-Sternberg cells in an
appropriate reactive cellular background.
-Hodgkin lymphoma has a bimodal age
distribution with one peak in the 20s and 30s,
and a second peak over the age of 50.
14.
15. Symptoms of hodgkin’s lymphoma:
• General symptoms:
I.Fever
II.Weight loss
III.Loss of appetite
IV.Night sweats
V.Pruritus
VI.lethargy
16. Hodgkin’s Lymphoma and skin
• Pruritus can be a symptom of a distinct
dermatologic condition or of an occult
underlying systemic disease.
• Hodgkin lymphoma is the malignant
disease most strongly associated with
pruritus, which affects up to 30 percent of
patients with the disease.
17. Hodgkin’s Lymphoma and Skin
• Pruritus can precede the clinical presentation of
lymphoma by up to five years and is often the
presenting symptom
• A brief review of the literature demonstrates that
the occurrence of hives as a presenting
manifestation of Hodgkin's disease is
uncommon though pruritus is known to be a
frequent symptom of lymphomas in general.
19. Hodgkin’s Lymphoma and Skin
• Skin lesions — A variety of skin
lesions have been associated with
Hodgkin lymphoma.
• These include ichthyosis, acrokeratosis
(Bazex syndrome), erythema multiforme,
erythema nodosum, necrotizing lesions,
hyperpigmentation, and skin infiltration
22. URTICARIA
• Urticaria involves intensely pruritic, raised
wheals, with or without edema of the
deeper cutis.
• It is usually a self-limited, benign reaction,
but can be chronic.
• Rarely, it may represent serious systemic
disease or a life-threatening allergic
reaction (around 1%)
23. Urticaria
• Triggers often can be identified in patients with
acute urticaria, although a specific trigger is
found in only 10 to 20 percent of chronic cases.
• Common triggers include allergens, food
pseudoallergens
• Urticaria can be caused by allergic reactions to
medications, especially antibiotics, and through
direct mast cell degranulation by some
medications, including aspirin, nonsteroidal anti-
inflammatory drugs, radio-contrast dye, muscle
relaxants, opiates, and vancomycin.
24. Urticaria
• Systemic disease is a relatively rare cause, with
the exception of Hashimoto disease; thyroid
autoimmunity may be associated with up to 30
percent of chronic urticaria cases
• Systemic illnesses that have been associated
with urticaria or angioedema include
mastocytosis, systemic lupus erythematosus,
vasculitis, hepatitis, and lymphoma.
25. Urticaria
• A wide variety of different infections,
including hepatitis , infectious
mononucleosis , Helicobacter pylori
infection , dental infections, sinusitis and
urinary tract infections, have all been
implicated as causing urticaria.
26. Kikuchi fujimoto disease
• Kikuchi-Fujimoto disease is a histiocytic
necrotizing lymphadenitis which is a rare and
benign condition
• Can mimic other diseases such as lymphoma,
tuberculous adenitis, metastatic disease, SLE,
cat scratch disease and infectious
mononucleosis
• pathogenesis is unclear but is believed to be an
immune response of T cells and histiocytes to
an unknown inciting agent such as EBV, HHV 6
& 8, HIV, toxoplasma and paromyxoma viruses
27. Kikuchi fujimoto disease
Yoshino T, Mannami T et al reported on
Two cases of histiocytic necrotizing
lymphadenitis (Kikuchi-Fujimoto's
disease) following diffuse large B-cell
lymphoma.
28. Kikuchi fujimoto disease
• Krueger GR, Huetter ML, Rojo J, Romero M,
Cruz-Ortiz H et al reported on Human
herpesviruses HHV-4 (EBV) and HHV-6 in
Hodgkin's and Kikuchi's diseases and their
relation to proliferation and apoptosis.
• Menasce LP, Banerjee SS, Edmondson D,
Harris M et al reported on Histiocytic necrotizing
lymphadenitis (Kikuchi-Fujimoto disease):
continuing diagnostic difficulties
29. Kikuchi fujimoto disease
• Immunohistochemical monitoring of
plasmacytoid cells in lymph node sections of
Kikuchi-Fujimoto disease by a new pan-
macrophage antibody Ki-M1P These results
may represent an additional argument favoring
the histiocytic origin of plasmacytoid cells.
• Additionally, they may point to an
immunohistochemical tool that facilitates the
differential diagnosis between Kikuchi-Fujimoto
disease, especially in early stages of the
disease, and malignant lymphoma.
31. Cholestatic pruritus
• Primary biliary cirrhosis, primary
sclerosing cholangitis, B and C viral
hepatitis, autoimmune hepatitis,
carcinoma of bile ducts, alcoholic cirrhosis
• Pruritus is caused by the bile acids in the
blood (cholemia) or skin
• Commonly treatment is with phototherapy,
cholestyramine, plasmapheresis
32. Pruritus in chronic renal failure and
dialysis patients
• uremic pruritus is due to Iron deficiency, release
of histamine, disturbances of calcium-phosphate
metabolism, secondary hyperparathyroidism,
proliferation of mast cells in the skin,
hypervitaminosis A
• Treatment of renal pruritus is based on the use
of ultraviolet therapy, emollients, activated
charcoal, cholestyramine, and phosphate
binding agents. Sometimes parathyroidectomy is
necessary for reduction of itching
33. Pruritus in hematologic diseases
• In polycythemia vera, where overproduction of
all three hematopoietic cell lines occurs, itching
may appear following contact with water or after
a hot bath
• It is caused by release of histamine and other
substances from an increased number of blood
basophils; antihistamines do not relieve from this
symptom, and currently the most effective
method to treat this kind of pruritus is the use of
salicylates, photochemotherapy or interferon-a
34. Pruritus in hematologic diseases
• In older patients the cause of pruritus can
be a malignant tumour, that may also lead
to anemia.
• Pruritus may also be present in patients
with hemochromatosis where the levels of
iron in blood and tissues are elevated
35. Endocrine pruritus
• Pruritus is present occasionally in diabetics.
• Itch may be generalized or more frequently
localized on the scalp, the genitalia or the
perianal area.
• It may be attributed to a concomitant candidiasis
or, more often, to poor control of diabetes,
sometimes expressed as elevated glycosylated
hemoglobin blood levels
36. Endocrine pruritus
• The pruritus in diabetes mellitus may also
be linked to neuropathy, dry skin, and drug
administration. Treatment consists in the
control of diabetes, and the use of
antifungal agents
37. Endocrine pruritus
• Pruritus and even chronic urticaria may be
associated with the presence of thyroid
autoimmunity and antibodies against several
thyroid components such as are thyreoglobulin,
and TSH receptor. Levothyroxin is the
appropriate treatment in such cases.
• Hormonal deficit in women in the
postmenopausal period may provoke vulvar
pruritus
38. Pruritus and malignancy
• Carcinoma of the lung, stomach, colon,
prostate, breast and pancreatic rarely
have been associated with generalized
pruritus
• Pruritus is an important symptom in
patients with different forms of
mastocytosis: solitary mastocytoma,
urticaria pigmentosa, systemic
mastocytosis
39. Pruritus and malignancy
• In carcinoid syndrome pruritus is
sometimes associated with flushing. The
pruritus is elicited by serotonin and
treatment with antiserotonin drugs
alleviates the symptom.
• The association of pruritus with tumors is
not always understood. It may be triggered
by immunological mechanisms, toxic
metabolites, iron deficiency, and dry skin
40. Drug-induced pruritus
• Morphine, opioids, angiotensine
converting enzyme inhibitors, analgesics,
vitamin A, contrast media, gold,
chloroquine and sulfonamides are among
the drugs that may induce pruritus
41. Pruritus and Hodgkin’s Disease
• About 30% of the patients with Hodgkin’s
disease feel itchy. Pruritus can be an early or
presenting complaint.
• It can be very severe, and this may imply a
worse prognosis. Hodgkin’s pruritus improves
after radiation therapy or chemotherapy
• Released from circulating basophils histamine
and leukopeptidase - from white blood cells -
may trigger pruritus associated with lymphomas
and leukemias
42. In conclusion
• The most important tool for treatment of
the pruritus in internal diseases is the
specific treatment of the concrete internal
disease, which should not be
underestimated
Editor's Notes
Hodgkin's lymphoma, although considered separately within the WHO classifications, is now recognized as being a tumour of, albeit markedly abnormal, lymphocytes of mature B cell lineage
