Hypopigmentation in an African Child

Katie Ratzan, a third year Dartmouth Medical School student serving as a Schweitzer Fellow at the Schweitzer Hospital in Gabon, Africa, would like help and advice.
" I would like to ask your help with a six year old girl who presented to our clinic at the Hopital Schweitzer, with her father & aunt. The child has a recent onset of hypopigmentation of the left side of her face & neck. As of six to seven weeks ago, her skin was entirely normal. This change in skin color progressed over the past six weeks. It is asymptomatic. She has had no constitutional symptoms. She was not sick during the months/weeks prior to the color change, did not take any medications prior to the skin change, did not travel, did not have an accident with any sort of chemical, does not use anything on her face (i.e. cremes, etc.). No one else around her has anything like this. No one else around her is sick. She's never had this before. She now puts some sort of indigenous healing/darkening creme on the spots on the back of her neck, which is why that is darker than the areas of her face.

By history, this started on her cheek and moved toward her nose. It stops abruptly at midline. It has since spread to her neck and scalp. It's macular/patch-like depending on the confluence of abutting lesions. There is no involvement of mucous membrance (mouth & vagina are normal). She has no trouble with vision, taste, hearing, and her neuro exam (my brief version of it which essentially only tested sensation and gross motor) was normal.





Questions from Katie:
1. Does anyone think this is anything other than vitiligo?
2. Is this segmental vitiligo, and if so what special significance does this have?
3. What therapy would be appropriate for a child like this in this setting?
4. What is known of the psychological and social implications of such hypopigmentation in a girl in this setting?

Thank you,
Katie

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One More Unfortunate...

Gina Kaulukukui is a grief counselor on the island of Kauai, Hawaii. She sent me the following text along with these photographs. Your impressions may help to solve this tragic mystery.

“I would like you to review the attached photos and give me your impressions. This 21 y.o woman was found in the water at Tunnels Beach on the north shore of Kauai following a night of partying. She was reportedly in the water about 7-15 minutes when she was pulled out unresponsive. When she was brought to the ER (at least a ½ drive) it was believed that she nearly drowned. She was unresponsive and not expected to survive.

While in the ER she presented on her thighs with unusual markings. The first appeared red jagged rings starting from her bikini line to just above the knee on one side and more toward the inner thigh on the other leg. Both were oval and completely blanched in the middle. There was a slight bruised color line in the center of one of the blanched areas. There were no others marks, lesions, etc.

The next day, the blanching went away and the red ring doubled in size from about 1 inch to about 2 inches. It was very red, raised and angry looking.

Later in the day when I went to check the young lady and convinced the ICU to photograph the area as it was again changing. Where the bruising was, small water blisters began to appear on the one leg, while the markings on the other leg was beginning to disappear. When the doctor was called to look at the blistering, she diagnosed it as a thermal burns.

The attached pictures were taken few hours following her death and as you can see on one side there is no marking left (the red mark on the upper thigh is from her blood pooling and not the original location of the blanching). The other side speaks for it self.

There were never any marks or puncture wounds that would indicate a jelly fish sting. The water was calm that day. She had high levels of cocaine and alcohol in her system. I would love to know what you think. We have yet to receive the biopsy results. I appreciate your time...love and aloha Gina

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Distal Onycholysis

Abstract: 76 yo retired nurse with a 1 year history of nail dystrophy.

History: This 76 yo retired registered nurse had distal onycholysis of her right thumb nail a little over a year ago. It eventually "spread" to involve all finger nails. Her medications include lorazepam, citalopram, Premarin and thyroid. All have been taken for many years. She has not used acrylic nails for more than five years. No unusual trauma, but she does use a nail file now. She was seen for around a year by a provider who was treating her with ciclopirox. The patient admits to being very anxious and plays with her nails.

O/E.: The patient is a pleasant, well-groomed woman who appears anxious and concerned. She has distal onycholysis of all finger nails. Toe nails are normal. Scant subungual debris.
Clinical Photos:




Lab: Three KOH preps negative. Fungal cultures were obtained 30 July, 2008.
Pathology: A "few" fungal elements were reported on PASD stained clipping of an affected nail

Diagnosis: Distal onycholysis. I am leaning away from onychomycosis. This would be an unusual presentation. I think this will likely be traumatic onycholysis.

Therapy: Pending culture report, I initiated therapy with 15% sulfactamide in ethanol twice daily. She was asked not to use a nail file and to clip separated portions of nails every day or so. Also, keep hands out of water as much as possible.

Questions: What are your thoughts? Any further work-up?

References:
1. eMedicine.com

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Skin Cancer Observation from Baghdad

Case presentation by:
Professor Khalifa Sharquie,
Baghdad, Iraq

I have had the opportunity to see many cases of skin grafting on the face after excision of multiple skin solar keratosis and skin malignancy. Some of these have been in patients with xeroderma pigmentosa (XP). The grafts remained free of actinic disease and have stayed clear for many years, in some cases for more than 20 years. I have never observed them to develop solar damage, solar keratosis or malignancy.

Today, I am presenting one of these cases. A 65 yo man with history of marked sun damage since early life. During the course of his illness, he has developed frequent and multiple solar keratosis and squamous cell carcinoma. Positive family history was seen in his son. Excisions and graftings have been carried out for big cancers since 1982 but he has never developed any solar damage or skin malignancy in the grafts.





Questions:
1. Is it justifiable to excise the skin of such patients with multiple keratosis and malignancies, especially in patients with XP early in life as a part of preventive measures against skin malignancy especially malignant melanoma.
2. What is the mechanism behind this odd observation. Could fibroblasts of the graft share in prevention?
3. Is there any role in the use of imiquimod in these patients? (last question from DJ Elpern)

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Erythema Migrans with Pustules

This 27 yo woman was sent from the E.R. with a 5 day history of a solitary 20 cm annular plaque on the right hip. It looks classical for erythema migrans (EM) of Lyme disease. Her three year-old daughter had a tick on her skin 2 weeks ago, but she has no history of tick bite. The patient is well otherwise.
Photos taken June 24, 2008


Clinically, this is Lyme disease, but no reports in literature of pustules. There is one from China with vesicles (which these may have been initially).

Lyme titers are pending (but this is a clinical dx at this point) and a culture of a pustule was taken.

E.R. doc put her on cefuroxime because she can't tolerate doxycycline (bad GI upset). She has a f/u appointment in a week.

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Ranula

Abstract: 16 mo girl with mucous cyst of lower lip.
HPI: This 16 mo Chinese girl presented for evaluation of a lip lesion that has been present for two months. It waxes and wanes in size. The lesion does not appear to bother her. Her mother speaks little English and is very worried about this lesion.
O/E: 6 mm translucent cyst lower lip
Clincal Photo:

Lab/Path: N/A
Diagnosis: Mucous Cyst (Ranula)
Questions: How would you approach this patient? I need to find a translator so that I can have a meaningful discussion with the child's mother. In the past, I have treated a few of these with liquid nitrogen and they did well, but that might be very traumatic for this child (and the mother). Might have to find a pediatric ENT (the closest would be ~ 75 miles from here)

Reference: There are two good chapters on eMedicine.com
and eMedicine2.com The latter is more detailed.

This is the largest study I found, and I'll write to the authors.
Clinical review.com2 of 580 ranulas.
Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2004 Sep;98(3):281-7.
Zhao YF, Jia Y, Chen XM, Zhang WF.

CONCLUSION: Three patterns of ranula have similar clinical and histopathologic findings, although plunging ranula has some different clinical features. Removal of the sublingual gland via an intraoral approach is necessary in the management of various clinical patterns of the ranula. Recurrence rates of ranulas of any type are excessive unless the involved sublingual gland is removed.

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Man from Mauritius

Presented by Dr. Philip Li Loong, Quatre Bornes, Mauritius

Abstract: 27 year old man with four year history of a papular eruption.

HPI: This man's lesions began on the face four years ago and later developed in the groins and axillae. Initially macules, they became papular and much more numerous. At onest, they were felt to be verruca plana. Cryotherapy was tried without success.

O/E: The lesions are 2 - 3 mm in diameter brown to yellowish papules. Distribution: Face, crural folds, axillae.

Clinical Photos:





Lab: Full blood count normal, cholesterol 5.2 upper limit, triglycerides normal, LDL HDL also normal, Immumoglobulins normal, plasma electrophoresis normal, thyroid function tests normal.
Pathology: Two biopsies were done.
The first showed a mild chronic inflammatory infiltrate in the upper dermis made up of small lymphocytes and histiocytes with occasional macrophages.
Repeat biopsy from the right axilla was reported as aggregates of foam cells admixed with other histiocytes, lymphocytes and some fibroblasts: "appearance consistent with eruptive xanthomas"

Diagnosis: Do you think this man has xanthoma disseminatum?

Questions: This entity may be associated with paraproteinemia and mucous membrane involvement. Comments for diagnosis and management will be most welcome. What are your thoughts? What further would you do?

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