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.
References:
1. eMedicine.com: 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. hajrei@uniklinik-saarland.de

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.

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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.

4. 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.

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Acanthosis Nigricans in a Child

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 - 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.
References:
1. eMedicine.com

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.

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Hypopigmentation in a Young Child

Presented by Dr. Henry Foong
Ipoh, Malaysia

History: A 6 year old boy presented with patches of hypopigmentation on the trunk and extremities since one year's of age. It was occasionally pruritic. He is otherwise well and has no history of fever or other constitutional symptoms. There was no family history of similar problem and no personal or family history of atopy.

Examination showed the skin was quite dry. Multiple irregular patches of hypopigmentation 2-4 cm diameter were distributed over the back of trunk involving both the lower back extending to the gluteal areas, the arms and anterior chest wall. Sensation was normal in the affected areas.

Photos:

Lab: KOH examination did not reveal hyphae or spores.
Pathology: Biopsy not performed as yet.

Diagnosis: ? Progressive Macular Hypopigmentation ? Pityriasis Alba

Questions: Have you seen this in a young child?? What is your diagnosis and therapeutic recommendations.

Reference:

Progressive macular hypomelanosis: an overview.
Relyveld GN, Menke HE, Westerhof W.
Am J Clin Dermatol. 2007;8(1):13-9.
The Netherlands Institute for Pigment Disorders, Academic Medical Center,
University of Amsterdam, Amsterdam, The Netherlands.

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Distinctive Disorder

The patient is a seven-year old girl with a one year history of a linear band of confluent hypopigmented scaly papules. She has Fitzpatrick Skin Type IV.



The clinical diagnosis is Lichen Striatus.

Reference: You can read a good chapter about L.S. on: Emedicine.com

Questions: This does not particularly bother the child or her parents. Would you treat this? And if so with what? A topical corticosteroid? Pimecrolimus? Tacrolimus? Other?

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Clam Digger's Legs

Mike LaCombe, a cardiologist from Maine, has a question for our panel.

"G.M. is a 48 y.o. clam digger from Maine. These individuals are exposed to avian schistosomiasis, and on a web search, the consequent clam-digger’s itch has been rarely associated with lymphedema.

Sorry, I have no pictures. This man has well-documented lymphedema of the lower extremities for six months.

All studies negative: CT scans of pelvis, abdomen, chest, ultrasound of leg veins, cardiac echo ruling out any cor pulmonale, and labs showing no evidence of liver disease, hypoalbuminemia.

My questions are:
1. Does anyone have any experience with this?
2. Any specific tests to confirm the diagnosis?
3. Therapy suggestions? "

There are more things in heaven and earth, Horatio, than are dreamt of in your philosophy." Always something new.

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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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