Acanthosis Nigricans

Abstract: 11 yo girl with three year history of acanthosis nigricans
History: This is a healthy 11 y.o. girl. Her mother noticed gradual darkening of skin in neck folds, axillae and groin around three years ago. The child is Chinese. Has not had her first menstrual cycle yet, although has some breast development. She is mildly overweight (not obese). Fitzpatrick Skin Type IV. No hirsuitism.
O/E: Velvety hyperpigmentation of skin folds. There are a few skin tags in axillae.
Clinical Photo:

Lab: Insulin Level 43 (normal 3 - 28) Hgb A1C (normal) , Serum Testosterone Level 75 (normal < style="font-weight: bold;">Histopathology: N/A
Diagnosis or DDx: Acanthosis Nigricans
Questions: How would you approach this patient and initiate an appropriate work-up?
For a full discussion go to: VGRD Blog

A Destinctive Dermatosis

A seven year-old girl presents with a one year history of a hypopigmented streak on the lower face.



What are your thoughts?

For answer go to VGRD Blog

The Ugly Duckling

Presented by Helge Riemann and David Elpern

Abstract: 12 yo boy referred for evaluation of nevi
History: The boy's pediatrician referred him for evaluation of nevi. There is no pertinent family history since he is adopted. His mother feels the large lesion on the back has changed over the past year.
O/E: Healthy child. Type II skin. All nevi, except one, look similar. The "outlier lesion" measures 1 cm in diameter, has variations in color and a white area.
Clinical Photo(s):


Dermoscopic Image

Lab: N/A
Histopathology: N/A
Diagnosis or DDx: Probable Dysplastic Nevus Syndrome in a 12 yo boy.
Questions: What would you do here?
Reason(s) Presented: To discuss the concept of the "ugly duckling" (outlier lesions). Lesions which stand out as not belonging with the others a patient has deserve more attention and one should have a lower index of suspicion for biopsying.

Discussion: Dr. Riemann
Dysplastic melanocytic nevi (DMN) are a heterogenuous group of nevi with atypical clinical features, histopathologic abnormalities, or both.
The clinical features of DMN include asymmetry, varying sizes frequently larger than regular melanocytic nevi, irregular or ill-defined borders, some color variegation showing two to three shades of brown, pink, skin-colored, or red, and surface irregularities like "cobblestoning" or "fried-egg" appearance. Histopathologically, DMN can show lentiginous proliferation, elongated or bridged rete ridges, nests varying in size, nest located in papillary tips, a junctional component extending beyond the dermal component, and some degree of cytologic pleomorphism.
Several studies have shown that the risk of melanoma is directly related to the number of ordinary nevi and to the number of DMN as defined by the clinical criteria above.
Although the term "dysplastic nevus syndrome" lacks a stringent definition, it is ususally used to describe an autosomal dominant condition of increased number of ordinary and DMN. Affected individuals have an increased risk to develop melanoma and therefore, require regular skin checks. As these patients, by definition, can have many clinically atypical nevi, the challenging task for the clincian is to identify any lesions suspicious for melanoma.
The "outlier" concept describes a helpful rule of thumb to look for any DMN which appears to clearly stand out as being different from the average, baseline clinical appearance of the patient's other nevi. These "outlier lesions" should be carefully evaluated for removal. Lesions suspicious for melanoma should then be removed with a 2-5 mm margin when possible
.
References:
1. Grob JJ, Bonerandi JJ.
The 'ugly duckling' sign: identification of the common characteristics of nevi in an individual as a basis for melanoma screening. Arch Dermatol. 1998 Jan;134(1):103-4

2. Dixon AJ, Hall RS. Managing skin cancer--23 golden rules. Aust Fam Physician. 2005 Aug;34(8):669-71.
From their collective experience in Australia and the USA, dermasurgeons Anthony Dixon and Scott Hall have compiled a list of "golden rules" for general practitioners to help reduce errors and problems with skin cancer management. It is anticipated that these tips will provide a brief yet informative reference when faced with skin cancer management concerns in general practice.
This valuable refrence can be viewed as FULL TEXT HERE

3. Emedicine.com has a good chapter on Dysplastic Nevi.

Abstract: 9 yo girl with vascular lesion of eyelid
History: A 9 yo girl has a one month history of a papule on left lower lid. It has bled on one occasion. She saw her pediatrician who referred her to dermatology.
O/E: 5 mm red papule
Clinical Photo(s)


After procedure:

Lab: N/A
Histopathology: Pending
Diagnosis or DDx: Pyogenic granuloma
Treatment: The area was anesthetized with lidocaine + epi and the lesion was snipped off with a curved iris scissors. The base was gently cauterized. Patient's father held her hand during the procedure.
Questions:
Reason(s) Presented: This is the kind of case that FPs can handle in the office. It akes a few minutes to reassure the patient, and it's important to have a parent there. Often it can take weeks to see a dermatologist or ENT or ophthalmologist. The procedure is simple. These lesions can reoccur after electrodessication, and if so may require excision. Imiquimod to the base may prevent that.
References: Pyogenic granuloma in children: treatment with topical imiquimod.
Fallah H, Fischer G, Zagarella S.
Australas J Dermatol. 2007 Nov;48(4):217-20.
We report the successful treatment of five children with facial pyogenic granuloma using topical imiquimod 5% cream. In all cases, resolution of the lesions was achieved within 2-4 weeks. Local erythema and scaling, consistent with a typical imiquimod response, was the most commonly observed side effect. No systemic complications were observed in any of the patients. There has been no recurrence of any of the lesions to date. Small mildly erythematous or hypopigmented macules remain at this stage of follow up.
[Had I read this first, this might have been a better approach!! But the combination may be easier as it was very quick]

Scalp Lesion in a Child

Abstract: 14 yo boy with alopecic area since birth
History: Shortly after birth the mother noted a yellowish alopecic area on the scalp. She was told it was from a scalp electrode and thought no more about it. About a year ago, the lesion became more raised and the surface pebbly (bossilated).
O/E: Five cm oval tan/pinkish alopecic plaque with a slightly bossilated surface.
Clinical Photo(s):

Lab: N/A
Histopathology:The epidermis shows papillomatous hyperplasia. In the dermis, the numbers of mature sebaceous glands are increased. Ectopic apocrine glands are often found in the deep dermis beneath sebaceous glands.

Diagnosis or DDx: Nevus Sebaceous of Jadasshon
Reason(s): Presented: You will all see these lesions. It is satisfying to give parents a good explanation and possibly a reference (#1) to read.
References:
1. Dermnet
2. eMedicine
3. Cribier B, Scrivener Y, Grosshans E.
Tumors arising in nevus sebaceus: A study of 596 cases.
J Am Acad Dermatol. 2000 Feb;42(2 Pt 1):263-8.
The rate of malignant tumors arising on NS was very low and we did not observe such cases in children, who had associated benign tumors in only 1.7% of cases. Benign neoplasms were common and most of them occurred on the scalp; this was not a bias resulting from a longer duration before surgery. Because most tumors occurred in adults older than 40 years, our study suggests that prophylactic surgery in young children is of uncertain benefit. Clinical follow-up is probably sufficient, and even those cases with clinical changes often proved to be benign tumors or warts.

4. Santibanez-Gallerani A, Marshall D, Duarte AM, Melnick SJ, Thaller S.
Should nevus sebaceus of Jadassohn in children be excised? A study of 757 cases, and literature review.
J Craniofac Surg. 2003 Sep;14(5):658-60
The incidence of basal cell carcinoma and the need for prophylactic excision in children with nevus sebaceus of Jadassohn have been a topic of controversy. The authors performed a retrospective analysis of 757 cases from 1996 to 2002 in children aged 16 years or younger. No cases of basal cell cancer were found in the nevus sebaceus group. Recent studies in children corroborate these findings and question the need for prophylactic surgical removal of the nevus sebaceus.

Take-Home Points:
1. Most sebaceous nevi will have a benign behavior. There is no crying need for excision at a young age.
2. These lesions have a characteristic appearance and can be diagnosed clinically.
3. They get thicker and more irregular at puberty as a result of hormones acting on the sebaceous glands

Two additional patients seen in Williamstown with N. sebacsous

Bizarre Hyperpigmented Streaks

Abstract: 30 y.o. man with three week history of hyperpigmented streaks over hip

History: The patient's brother was seen for an unrelated problem and asked if I would look at a picture on his cell phone. His brother, a 30-year-old man, recently returned from the Bahamas where he developed a pruritic inflammatory skin disorder. The cell phone image was hazy, but interesting and I asked if his brother could come in that day. He did, and anamnesis revealed that he had been drinking Corona beer with lime and remembers rubbing his abdomen at that time. Twenty-four to 36 hours later, he developed blisters and erythema at the site where he rubbed his skin. He thought it was from the limes, but his friends told him this was fanciful.

O/E: The examination showed streaky erythema on the right hip. The lesions are along the lines of where he rubbed with his fingers.
Clinical Photo(s)
Brother will cell-phone


Patient demonstrating how he remembers rubbing his abdomen.



Lab: N/A
Histopathology: N/A
Diagnosis or DDx: Phytophotocontact Dermatitis due to Lime (Rutaceae)
Questions: N/A
Reason(s) Presented: Presented for interest. It would be possible for a patient to send an image to his dermatologist from the Bahamas on his cell phone and a tentative diagnosis arrived at. Knowing that limes contain furocoumarin (a potent photosensitizer) could have led to a more timely diagnosis. If treatment was initiated very early, some of the post-inflammatory hyperpigmentation might have been avoided. At that time, wet compresses and topical corticosteroids could have shortened the course of the inflammation.
Discussion: See reference. Limes, lemons and a number of other plants contain furocoumarin. To develop phytophotocontact dermatitis, an individual needs to have contact with the agent and then get sun-exposure before washing the skin. Then, 24 - 36 hours later an exaggerated sunburn develops which heals with post-inflammatory hyperpigmentaton. The pigment change is more pronounced in darker individuals and can last many months. It usually fades over time.

Reference: Hyperpigmented Macules and Streaks

Dermatologic Vignette

Abstract: 75 yo woman with 3 week history of a rash.
History: Painful rash in groin. Healthy woman. Was on antibiotics around a month ago. She used a topical cream given for another purpose by her Gyn. (It turns out the cream she had been using was clobetasol -- a super potent corticosteroid)
O/E: Firey erythema of crural folds, pubic area. Studded with fine superficial pustules.
Clinical Photo(s)

Lab: KOH prep from pustule loaded with budding heasts
Histopathology: N/A
Diagnosis or DDx: Candidiasis (Candidal Intertrigo: iatrogenic)
Reason(s) Presented: For interest. Rick factors for candida intertrigo include antibiotics, diabetes, topical and oral steroids, OCP, pregnancy. This patient took antibiotics and was applying a superpotent corticosteroid to the area. The latter acts as fertilizer for the yeast.
Reference: emedicine.com