A 28-year-old female, experiencing skin rashes and bilateral knee pain from past 15 days, presented to the clinic

Case quiz study - 27/07/2022

Case quiz study exposes you to a step-by-step analysis of the case discussed.
The MCQs poser is placed in critical junctures where the reader can exercise his thoughts in diagnosis by answering them.
It’s designed to help the reader to learn about the clinical features, investigations to be performed, any clinical interventions, and how to conclude the diagnosis
Take the quiz to enhance your ability to diagnose the case.

Note Correct answers and Discussion of the case from an expert are available after completing all the Questions.

1 / 6

What are the probable diagnosis to be considered except

Thrombocytopenic lesions

Henoch-Schönlein purpura

Allergic-leukocytoclastic vasculitis

Psoriatic arthritis

2 / 6

The above images of both legs and elbows indicates

Thrombocytopenic lesions

Psoriatic scaly lesions

Multiple erythematous maculopapular rashes

urticaria

3 / 6

The thrombocytopenic cause is excluded due to

Normal platelet count

No bleeding

Both a and b

None of the above

4 / 6

What are the further test recommended ?

ANCA, Complement C3-C4 levels, ENT evaluation, chest and PNS X-rays

Complement C3-C4 levels, ENT evaluation, chest X-ray

ENT evaluation and chest X-ray

Chest and PNS X-rays

5 / 6

What is the final diagnosis of the case?

Wegener's Granulomatosis

Henoch-Schönlein purpura

Allergic-leukocytoclastic vasculitis

ANCA associated cutaneous vasculitis

6 / 6

Henoch-schönlein purpura can be excluded as the following criteria is not fulfilled

Development of first symptoms at age 20 years or less

Diffuse abdominal pain worse after meals, or bowel ischemia, usually bloody diarrhea

Histological changes showing granulocytes in the walls of arteries or venules

All the above

Case discussion

The 28-yr-old female had a 15-day history of gradual onset of erythematous petechial rashes, predominantly in lower limb and abdomen. History revealed that the rashes appeared in crops and faded in 3-4 days, however some were ulcerative and non-itchy. Clinical investigation revealed that the patient was positive for ANA, ANCA positive and anti-PR3. The lesions were raised, petechial, purpuric and ecchymosis. The complement levels were normal. CRP and ESR were elevated.

The prominent clinical presentation in the present case was purpura. The purpuric lesions should be differentiated from thrombocytopenic/non-thrombocytopenic lesions. The probability of thrombocytopenic causes could be excluded, since the platelet counts were normal and no associated features of bleeding were reported. Non-blanching of the lesion, while pressing, indicated that it wnnjmas extravascular. Purpura may be classified as: palpable and non-palpable. Non-palpable purpura is caused by blood leaking from a normal blood vessel due to trauma or a hematologic problem like thrombocytopenia, coagulation defects or capillary fragility. While palpable purpura causes are inflammation around the blood vessels. The differential diagnoses are vasculitis and an inflammation due to infection around the blood vessels. Henoch-Schönlein purpura (HSP)is common in children, while vasculitis is common in adults (rarely allergic-leukocytoclastic vasculitis). These lesions represent small vessel vasculitis of various causes. Rarely, the vasculitis may not be palpable in the early stages, and may show blanching (Riordan, 1996). Petechial lesions restricted to the area above the clavicles signify a benign cause, generally accompanied by coughing or vomiting. Various causes of purpura are listed in table 1.

Table 1: Categorization of various causes of purpura

The palpable and non-blanching rashes noted in the current case suggest the possibility of vasculitis or allergic rashes. The presence of arthralgia and a few ulcerating rashes indicated the possibility of vasculitis. The non-ulcerative palpable lesions are small vessel vasculitis, while ulcerating lesions are medium to small vessels. Clinical clues, which may help in categorizing the size of the vessels involved, are given in below table 2.

Clinical features	Most likely affected vessels	Most commonly associated systemic vasculitis
Cutaneous
Palpable purpura	Post-capillary venules	Any type of vasculitis except giant cell arteritis and Takayasu's arteritis
Skin ulcers	Arterioles to small arteries	Polyarteritis, Churg-Strauss vasculitis, Wegener's granulomatosis, hypersensitivity vasculitis
Gangrene in an extremity	Small to medium-sized arteries	Polyarteritis, Churg-Strauss vasculitis, Wegener's granulomatosis
Gastrointestinal tract
Abdominal pain or mesenteric ischemia	Small to medium-sized arteries	Henoch-Schönlein purpura, polyarteritis, Churg-Strauss vasculitis
Gastrointestinal bleeding	Capillaries to medium-sized arteries	Henoch-Schönlein purpura, polyarteritis, Churg-Strauss vasculitis
Renal
Glomerulonephritis	Capillaries	Microscopic polyangiitis, Wegener's granulomatosis, cryoglobulinemia, Churg-Strauss vasculitis, Henoch-Schönlein purpura
Ischemic renal failure	Small to medium-sized arteries	Polyarteritis, Takayasu's arteritis; less commonly, Churg-Strauss vasculitis and Wegener's granulomatosis
Pulmonary
Pulmonary hemorrhage	Capillaries; less commonly small to medium-sized arteries	Microscopic polyangiitis, Wegener's granulomatosis
Pulmonary infiltrates or cavities	Small to medium-sized arteries	Churg-Strauss vasculitis, microscopic polyangiitis
Neurologic
Peripheral neuropathy	Small arteries	Polyarteritis, Churg-Strauss vasculitis, Wegener's granulomatosis, cryoglobulinemia
Stroke	Small, medium-sized or large arteries	Giant cell arteritis, SLE-associated vasculitis

The probable diagnoses are: HSPor hypersensitivity vasculitis (HSV), drug-induced vasculitis, and other primary vasculitis including microscopic polyangiitis; among infections rickettsial infections, viral infections and streptococcal infections; secondary causes of vasculitis including lupus. The presence of ulcerating lesions is not commonly seen in HSP and HSV. ACR classification criteria for HSP are given in table 3.

Table 3: ACR classification criteria for HSP

Criterion	Definition
Palpable purpura	slightly elevated purpuric rash over one or more areas of the skin not related to thrombocytopenia

Age at onset	< 20 years Development of first symptoms at age 20 years or less

Bowel angina	Diffuse abdominal pain worse after meals, or bowel ischemia, usually bloody diarrhea

Wall granulocytes on biopsy	Histological changes showing granulocytes in the walls of arteries or venules

Note: For the purpose of classification, a patient shall be said to have HSP if at least 2 of these 4 criteria are present. The presence of any 2 or more criteria yields a sensitivity of 87.1% and specificity of 87.7%

It is important to consider the possibility of other vasculitis and other similar rashes. Clinical characteristics to be considered for differentiating different types of C- ANCA-positive vasculitis are listed in table 4.

Table 4: Clinical characteristics differentiating different types of C- ANCA-positive vasculitis

Conditions	Wegener's Granulomatosis	Microscopic Polyangitis	Polyarteritis Nodosa	Chrug Strauss Syndrome	Comments
Pulmonary infiltrates or nodules	+++	++	-	+++	Asthama and eosinophilia CSS
Alveolar hemorrhage	++	++	-	+
Glomerulonephritis	+++	+++	-	++	Progressive renal failure uncommon in CSS
Upper airway disease	+++	+	+	++	ENT disease usually favours WG
Skin / purpura	+	+++	-	++
Peripheral nervous system	++	+	++	+++	Often a prominent feature of CSS
Central nervous system	+	+	+	++

The patient did not fulfil the complete criteria for the diagnosis of Wegener's or any of the vasculitis. In addition, no systemic clinical features of definable syndrome of ANCA-associated vasculitis were reported. In order to confirm the diagnosis of ANCA-positive cutaneous vasculitis, skin biopsy of an active lesion, ENT evaluation for asymptomatic involvement, and chest and PNS X-rays to exclude the possible involvement of these organs were conducted.

Final diagnosis

ANCA-associated cutaneous vasculitis

Course of treatment

She was managed on small doses of steroids and azathioprine. The rashes reduced and symptomatically improved. The patient is on regular follow-up and there is no further organ involvement.

Your score is

A 28-year-old female, experiencing skin rashes and bilateral knee pain from past 15 days, presented to the clinic. The patient had an intermittent fever for the past 4 days. The fever was associated with chills, myalgia, headache, and sore throat. Complete this step-by-step quiz to find out the case study’s final diagnosis.

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