Perplexing Periodic Pruritus

This case was posted by David Elpern MD as a rapid publication for VGRD
We have been having technical problems with VGRD and have elected to publish this case here. Your comments will be appreciated -- please mail them directly DJ Elpern and we will post them to VGRD when it is up and running,

78 yo man with episodic incapacitating pruritus

Duration: 4 years

Distribution: Generalized, but sparing head and feet


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

O/E: Excoriated papules – some crusted. Arms, legs, lower back, sacrum – sparing mid back, face. No vesicles or bullae.

Bx:
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

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

DISCUSSION: All of his dermnatologists thought of D.H. but the biopsies have not confirmed this and dapsone and gluten free diet have not helped.

Please respond directly to DJ Elpern

Nail Abnormality in a young girl

This case was submitted by Dr Choon Siew Eng FRCP, a dermatologist from Johor Bahru, Malaysia

A 14-year-old Malay girl presented with a 7-month history of asymptomatic nail abnormality affecting all fingernails. Good general health. No previous of current skin problem or hair loss. No family history of skin problem. No personal or family history of atopy. No family history of autoimmune disease. No nail biting habit. Never varnish nails and no excessive wet work. No on any medication, traditional preparations or supplements.

All fingernails are abnormal ranging from mild horizontal lamellar dystropy, longitudinal, v-shaped, u-shaped and w-shaped sulci as shown in images with variable erythema at the edges of abnormalities. Majority of eponychium appeared normal. Lunula and periungual skin no capillary dilatation noted although some had streaky hyperpigmentation. No palmar erythema. Finger pulps, toe nails, scalp and oral mucosa normal. No other skin lesions

Laboratory: Fungal culture negative

Histopathology: Reject biopsy.

Diagnosis: Lichen Planus, Part of connective tissue disease,

Comments: Would appreciate help in diagnosis and suggestion on further investigations and management.

Questions: Am puzzled by how normal and healthy the unaffected parts of nails look. Had never seen such pattern of erythema. Have anyone seen similar abnormality? Is it justifiable to offer patient systemic steroid to prevent further nails damage and scarring without an histologic diagnosis?

Mistakes

Osler urged us to "Carry a small note-book, and never ask a new patient a question without note-book and pencil in hand......Begin early to make a three-fold category - clear cases, doubtful cases and mistakes. And learn to play the game fair, no self-deception, no shrinking from the truth; mercy and consideration for the other man, but none for yourself, upon whom you have to keep an incessant watch. It is only by getting your cases grouped in this way that you can make any real progress in your post-collegiate education; only in this way you gain wisdom with experience."

In this spirit, I present the case of G.K. an 82 yo woman who I have been following for 6 months for a pustulo-vesicular dermatitis of her feet. It had features of dyshidrotic eczema at only minimal control.

On August 31, 2007 the patient was reevaluated. Clinically and dermoscopically there was a suggestion of burrows and a scabies prep was taken. To my great surprise there were 4 - 5 mites and numerous eggs and feces in the mineral oil mount. She has no eruption of arms or torso and her only itching was on her feet.




I took this image from the web, but it shows what I saw -- a number of mites and eggs.

Diagnosis: Localized Norwegian Scabies. This patient is confined to a wheelchair, but is active and alert. Further history revealed that her grown children had scabies three years ago and all were treated (as was this patient) with 5% permethrin cream. Seemingly, they all got better, but then three years later this patient has a localized form of Norwegian scabies. There are only one or two case reports of localized Norwegian scabies, and none in patients like this.

Treatment: The patient, her husband and son who all live together will be treated with Elimite. She will have treatments every other day for two weeks for her feet and complete skin treatments twice, one week apart. She will be seen back in two weeks. A culture was taken for the question of secondary infection (this grew out coagulase sensitive Staph and she was placed on an appropriate antibiotic).

Over the years, I have been humbled by scabies time and again. Scabies localized to the plantar aspects of the feet is just the latest incarnation.

Addendum: Here is an article which addresses localized Nowegian scabies co-authored by the prolix Ted Rosen. Our patient is presumable immunocompetent.

Localised genital Norwegian scabies in an AIDS patient.
Perna AG, Bell K, Rosen T. Sex Transm Infect 2004;80:72-3.
OBJECTIVES: We present a case of an AIDS patient with Norwegian scabies manifest
by a single, crusted plaque localised to the glans penis. METHODS: A 45 year old
man with AIDS presented to our clinic complaining of a red papular pruritic rash
on his abdomen and anterior thighs and a single, thick, crusted, non-pruritic
lesion on the penis. He had been treated with lindane topically prior to the
development of the penile lesion without resolution of the pruritus or red
papular lesions. A mineral oil preparation was obtained from the hyperkeratotic
penile lesion and revealed numerous mite eggs and faeces. RESULTS: The diagnosis
of localised, genital Norwegian scabies was made. The patient was treated with
ivermectin 200 micro g/kg per dose taken as two doses, 14 days apart, with
complete resolution of both pruritus and skin lesions. CONCLUSIONS: This patient
is the first known report of Norwegian scabies localised as a single lesion on
the penis. He was successfully treated with oral ivermectin monotherapy.