Perplexing Periodic Pruritus

presented by

David J Elpern MD

Williamstown, Massachusetts, USA

September 29, 2007
 
Abstract 78 yo man with episodic incapacitating pruritus
Patient
78-year-old man
Duration
4 years
Distribution
Generalized, but sparing head and feet
History

The patient is a 78-year-old semi-retired music producer who presents with a four-year history of episodic intense pruritus that is generalized but has spared the face and the feet. It may have begun on the brachioradial areas of the arms but over a period of time spread to involve the sacrum, the chest, the back and the legs. He describes “a burning itch that you just cannot ignore.” At times he has “gone into a panic” over this. The itch can feel like “hot needles” applied to certain areas of the skin. He has also had scrotal pruritus for two years

The patient has seen a number of different physicians for this including four dermatologists. He has had biopsies done with nonspecific findings and direct and indirect immunofluorescence studies also have been noncontributory. There have been positive ANAs but no one has been able to correlate those with his pruritus.

His brother, has a similar problem and has been treated with CsA and azathioprine with good results.

Treatment:
1. multiple antihistamines - no help.
2. multiple topical steroids - no help.
3. Gabapentin - caused severe dizziness...unable to use.
4. prednisone, three courses - resolved or alleviated symptoms.
(though less completely the second and third time.)
5. plaquenil, one month - no help.
6. naltrexone, 2 weeks - no help.
7. dapsone, 2 weeks - no help.
8. pentoxifylline 400 qd x 2 weeks - no help.
9. cellcept 2000 mg x 19 days - no help.
10. Local heat hot compress seems to resolve it quickly though only temporarily.
11. Immuran
Physical Examination
Excoriated papules – some crusted. Arms, legs, lower back, sacrum – sparing mid back, face. No vesicles or bullae.
Images

Laboratory Data

nil
Histopathology

A superficial perivascular and interstitial lymphocytic infiltrate with scattered eosinophils and occasional neutrophils . Note : These changes are urticarial in nature. The histologic differential diagnosis includes urticaria or a dermal hypersensitivity reaction such as a drug eruption or insect bite reaction. Clinico-pathologic correlation is suggested.

 
Diagnosis

Uncertain. Possibly Subacute Prurigo
Reasons Presented

For diagnostic and therapeutic suggestions.. Have you seen similar cases? What has been the outcome?
Questions

All of his dermatologists thought of dermatitis herpetiformis but the biopsies have not confirmed this and dapsone and gluten free diet have not helped.

References


Keywords pruritus, prurigo
Comments from Faculty and Members

Jerome Z Litt MD, Assistant Clinical Professor of Dermatology, Case Western Reserve University School of Medicine, Cleveland, OH, USA on September 29, 2007

This disorder is, I trow, comparable to Jean-Paul Marat's intractable itching, during the French Revolution, where he was virtually confined to a bathtub for about 4 years.
I wrote a small article about it 30 or so years ago and suggested it might have been a variant of dermatitis herpetiformis.
Does anyone know if he routinely takes any medications? Vitamins? Supplements? Herbals?
Keep up the great site!

Rick Sontheimer MD, Professor and Vice-Chairman, Dept. of Dermatology University of Oklahoma Health Sciences Center, Oklahama City,OK, USA on September 29, 2007

Just to be on safe side I would first try an emperic trial of ivermectin for occult scabies (two treatments 1 week apart). What about his drug/supplement history? Then for subacute prurigo I would push the daily dose of Dapsone up to 200 mg/d or hematologic intolerance. Then I would go back to an extended trial of CellCept at 3,000 mg/day (at least 3 months).

Khalid Hawsawi MD, Consultant Dermatologist and Head, Department of Dermatology,King Abdul Aziz Hospital, Makkah, Saudi Arabia on Oct 4, 2007

In addition to your differential diagnoses, there are three very important diseases that should be ruled out. These are: Lichen planus, scabies and atopic eczema. Presence of family history support scabies and atopic eczema. Biopsy of a fresh lesion may help in diagnosing lichen planus. Empirical treatment of scabies may be warranted.

Davy Chan MD, Hong Kong, China on Oct 9, 2007

I can think about these two differentials: scabies and papular urticaria. On the other side of the coin, should the patient to see a psychiatrist, too ?

Frank Jonelis MD, Kaiser Hospital, San Francisco, CA, USA on Oct 9, 2007

Other thoughts you probably have ruled out are - Multiple sclerosis, Iron deficiency, internal malignancy and hepatobiliary disorder.

Azar Maluki MD, Asst Prof Derm and Ven, Kufa College of Medicine, Najaf, Iraq on Oct 10, 2007

Strict sparing of certain areas over years with such type of periodic itching makes scabies a very remote possibility. Involvement of the brother can raise diagnosis of atopic diathesis especially in advanced age where dry skin is a strong contributory factor.
I would treat such case by heavy topical lubrication with suitable doses of tranquilizers. Sometimes controlled doses of oral immuran could be helpful.

Please Click Here To Comment and Evaluate

Back to September 7, 2007 Case