Saturday, November 15, 2008

Pyoderma Gangrenosum

Abstract: 46 yo man with 1.5 year history of leg ulcers
History: The patient, a disabled 46 yo Cambodian man, has a four year history of poorly controlled ulcerative colitis. He has had painful leg ulcers for the past two years. These begin with pustules or vesicles by history. At present he is taking 1200 mg of Asacol t.i.d. and prednisone 30 mg. per day. In addition to the prednisone he has used potent topical steroids for his ulcers and has been treated at a wound care clinic.
Social History: The patient emigrated from Cambodia 25 years ago. He is married with three children and was employed until he became disabled 2 years ago from colitis and leg ulcers. His English is limited and I had no Cambodian translator.
O/E: There are two ulcers with raised overhanging borders on the left medial malleolus. In addition, there is post-inflammatory hyperpigmentation and proximal scarring secondary to previous ulcerations. The patient has Cushingoid facies.
Clinical Photos:

Lab: N/A
Histopath: N/A
Diagnosis: Pyoderma gangrenosum (P.g.)
Discussion: There is no effective therapeutic protocol for P.g. He has been treated with high dose prednisone for months and his P.g. is only poorly controlled. Super-potent topical steroids have been used without improvement. It seems to us that tacrolimus ointment should be tried because there are many reports of its efficacy with P.g. and it is a more benign therapy than oral cysclsporin or mycophenolate mofetil. Colectomy may be a more permanent solution, but the patient and his gastroenterologists are not ready for that.
Questions: Your suggestions are welcome.
1. P.G.

2. Reichrath J, Bens G, Bonowitz A, Tilgen W. Treatment recommendations for pyoderma gangrenosum: an evidence-based review of the literature based on more than 350 patients. J Am Acad Dermatol. 2005 Aug;53(2):273-83.

Dermatology Clinic, The Saarland University Hospital, Homburg/Saar, Germany.

Because the incidence of pyoderma gangrenosum (PG) is low, no prospective randomized controlled trials and only a few studies with case numbers of more than 15 patients have been published. To date no guidelines for treatment of PG have been established far. The aim of the study was to provide an evidence-based review of the literature and an evaluation of recommendations for PG treatment. We performed an electronic search using the PubMed database and the term "pyoderma- gangrenosum." Literature published in the English language during the past two decades was reviewed. All relevant studies that could be obtained regardless of the study design were evaluated for grades of recommendation and levels of evidence. Data on patient characteristics including severity of the disease, localization of lesions, associated diseases, and treatment procedures were abstracted and evaluated for therapeutic outcome. We conclude that therapeutic efficacy of systemic treatment with corticosteroids and cyclosporine is best documented in the literature for disseminated as well as for localized disease and should be considered first-line therapy. In cases that do not respond to this treatment, we recommend alternative therapeutic procedures (eg, systemic treatment with corticosteroids and mycophenolate mofetil; mycophenolate mofetil and cyclosporine; tacrolimus; infliximab; or plasmapheresis), considering additional factors including associated diseases.

Alopecia in a Child

This 11 yo boy has had this alopecic area since infancy. He has been with adoptive parents since he was a baby and his mother says this has been here since coming to live with her. At first, I thought this was a nevus sebaceous, but the scalp looks normal here with none of the raised "pebbly" surface seen in this disorders in older children.

My working diagnosis here is "Congenital Triangular Alopecia."

References: (supplied by Brian Maurer)
1. Elmer KB, George RM. Congenital triangular alopecia: a case report and review. Cutis. 2002 Apr;69(4):255-6.
Congenital triangular alopecia is a nonscarring loss of hair mass on the scalp's temporal regions. The area of hair diminution commonly is described as triangular or lancet shaped. Although previously considered congenital, this condition usually is noticed after 2 years of age and, more recently, is thought to be acquired. We propose that this entity be renamed triangular alopecia. Because this condition involves normal rather than inflamed skin, it does not respond to topical or intralesional steroids. It is important to make the correct diagnosis to avoid unnecessary and potentially harmful interventions. We present the case of a 10-year-old boy with triangular alopecia.

2. Congenital Triangular Alopecia occurring in sisters. Full Text. Original in Portugese

3. García-Hernández MJ, Rodríguez-Pichardo A, Camacho F. Congenital triangular alopecia (Brauer nevus). Pediatr Dermatol. 1995 Dec;12(4):301-3.Department of Medical-Surgical Dermatology and Venereology, Virgen Macarena University Hospital, Seville, Spain.
Abstract: Congenital triangular alopecia is manifested at 3 to 5 years of age by unilateral or, less frequently, bilateral patches of alopecia in the frontotemporal region. At this age the differential diagnosis is important, particularly as regards alopecia areata. Only about 47 cases have been reported, probably because the lesion is benign and nonprogressive. In 6200 patients seen in index visits, we found 7 with triangular alopecia, a frequency of 0.11%. We believe that males do not require treatment because of the later development of androgenic alopecia, but in women, surgical treatment is successful.2. Tosti A. Congenital triangular alopecia. Report of fourteen cases.

Tosti A. Congenital triangular alopecia. Report of fourteen cases. J Am Acad Dermatol. 1987 May;16(5 Pt 1):991-3.
Abstract: Fourteen patients affected by congenital triangular alopecia are presented. The clinical and histologic features of this condition are discussed. I suggest that the condition is considerably more common than hitherto has been thought.

Tuesday, November 11, 2008

Acanthosis Nigricans in a Child

Abstract: 11 yo girl with three year history of acanthosis nigricans

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 - V. No hirsuitism.
O/E: Velvety hyperpigmentation of skin folds. There are a few skin tags in axillae. Some perioral darkening
Clinical Photo:

Lab: Insulin Level 43 (nl 3 - 28)
Hgb A1C normal, Serum Testosterone Level 75 (normal < style="font-weight: bold;">Histopathology: N/A
Diagnosis or DDx: Acanthosis Nigricans
This child may have AN associated with obesity or a syndromic AN associated with insulin resistance. Too early to say if PCOS is related. We do not have any pediatric endocrinologists in our area, but I feel that she should travel to see one. Dr. Susan Ratzan has kindly given us some guidelines (see below)
Questions: How would you approach this patient and initiate an appropriate work-up? (See Dr. Ratzan's comments below) Is this high insulin level significant?
Reason(s) Presented: To Discuss implications of this diagnosis and work-up.

2. Hermanns-Lê T, Scheen A, Piérard GE. Acanthosis nigricans associated with insulin resistance : pathophysiology and management. Am J Clin Dermatol. 2004;5(3):199-203.
Departments of Dermatopathology, University of Liège, Liège, Belgium.

The association of acanthosis nigricans, skin tags, diabetes mellitus due to insulin resistance, and obesity in adolescents and young adults represents a well defined syndrome. Hyperandrogenism may also be present. The endocrine origin of this condition is beyond doubt. Insulin and insulin-like growth factor-1, and their receptors on keratinocytes are obviously involved in the complex regulations leading to the peculiar epidermal hyperplasia. This condition is unrelated to other types of acanthosis nigricans, including the congenital and the paraneoplastic types.Control of obesity contributes largely to reverse the whole process, essentially by reducing both insulin resistance and compensatory hyperinsulinemia. Several drugs including metformin, octreotide, retinoids and topical colecalciferol (vitamin D(3)) analogs are also beneficial in clearing acanthosis nigricans.

Comments by Susan Ratzan, M.D. (pediatric endocrinologist):
As far as I am concerned AN is the cutaneous manifestation of hyperinsulinism or insulin resistance. For starters I would get a very good family history for type 2 diabetes, PCOS, hirsutism, infertility, irregular menses, and obesity. You described her very politely as "chunky" but what is her BMI? The way I would document her degree of insulin resistance/carbohydrate tolerance is by doing a 2 hr oral glucose tolerance test with samples at 0, 30, 60, 90 and 120 minutes for BOTH insulin and glucose. At the 0 sample, since she will be fasting, I would also get cholesterol, LDLdirect, triglycerides and HDL(many of these children have the dyslipidemia associated with metabolic syndrome which is elevated TG and low HDL). The best treatment for insulin resistance, but the most difficult to achieve, is a healthier lifestyle, lots of fresh fruits and veggies, healthy oils, fat free milk, no fast food, no soda or other sugar sweetened beverages and juice limited to 4-6 oz/day. We also recommend limiting carbs to 5-6 servings(and they need to be taught what a serving is)/day. If only I could live like this!! Exercise needs to be worked up to an hour a day by turning off the TV and the video games/internet. If the child has glucose intolerance or severe insulin resistance, we use metformin even in children as young as 11, but nothing works as well as lifestyle change.