Onychomadesis

The patient is a 21 yo college student who emailed me around a month ago. He was away at school at the time:
Nov. 15, 2008 Dear Dr. Elpern, I was wondering if you had any idea what this skin rash / irritation is being caused by. On my hands and feet I've got these little red dots scattered all over. They don't itch, but offer a mild pain when applying pressure. Most of them are plush (sic) with the skin, but some of them are raised up slightly. Also my taste buds are inflamed and red... but I think this is an unrelated condition. Any help you could offer would be greatly appreciated.
He wrote back on December 20, 2008: Shortly after writing you the dots seemed to go away, so I didn't bother setting up an appointment; however, although the red dots went away, I did notice that the white half circle, that are supposed to be at the bottom of the nail, seemed to become weird and displaced on both middle fingers. About two weeks went by and nothing really changed. Yesterday things got worse. Both my middle finger nails seem to be falling off at their roots. I'm not sure what's causing this, and I was wondering if you thought I should set up an appointment, or if you think that I should seek help elsewhere.

O/E: The patient was seen on December 23, 2008: At this time, he had a separation of the proximal nail fold of both middle fingers. No other abnormal findings.

Clinical Photos:




Diagnosis: Post viral onychomadesis. The illness he had was most likely Hand, Foot and Mouth Disease or a related enterovirus infection. I have never seen nail dystrophy after this, but onychomadesis has been reported at least three times after similar episodes. One report is of an outbreak in Spain. I wonder if this is not another enterovirus infection.
Question: Has anyone else seen this?
References:
1. Salazar A, et al. Onychomadesis outbreak in Valencia, Spain, June 2008. Euro Surveill. 2008 Jul 3;13(27). pii: 18917. Available Full Text
2. Bernier V, Labrèze C, Bury F, Taïeb A. Nail matrix arrest in the course of hand, foot and mouth disease. Eur J Pediatr. 2001 Nov;160(11):649-51
Onychomadesis describes complete nail shedding from the proximal portion; it is consecutive to a nail matrix arrest and can affect both fingernails and toenails. It is a rare disorder in children. Except for serious generalised diseases or inherited forms, most cases are considered to be idiopathic. Few reports in literature concern common triggering phenomena. We present four patients in whom the same benign viral condition in childhood appeared as a stressful event preceding onychomadesis. In each case, spontaneous complete healing of the nails was achieved within a few weeks. CONCLUSION: Onychomadesis and/or onycholysis is a newly recognised complication in the course of viral infections presenting clinically as hand, foot and mouth disease, and because of mild forms, is probably underestimated.
Clementz GC, Mancini AJ. Nail matrix arrest following hand-foot-mouth disease: a report of five children. Pediatr Dermatol. 2000 Jan-Feb;17(1):7-11.
Hand-foot-mouth disease (HFMD) is a contagious enteroviral infection occurring primarily in children and characterized by a vesicular palmoplantar eruption and erosive stomatitis. Nail matrix arrest has been associated with a variety of drug exposures and systemic illnesses, including infections, and may result in a variety of changes, including transverse ridging (Beau's lines) and nail shedding (onychomadesis). The association of HFMD with Beau's lines and onychomadesis has not been reported previously. Five children, ages 22 months-4 years, presented with Beau's lines and/or onychomadesis following physician-diagnosed HFMD by 3-8 weeks. Three of the five patients experienced fever with HFMD, and none had a history of nail trauma, periungual dermatitis, periungual vesicular lesions, or a significant medication intake history. All patients experienced HFMD within 4 weeks of one another, and all resided in the suburbs of the Chicago metropolitan area. In all patients the nail changes were temporary with spontaneous normal regrowth. The mechanism of the nail matrix arrest is unclear, but the timing and geographic clustering of the patients suggests an epidemic caused by the same viral strain.

Comment: It is likely that this young man's nails will regrow. However, it may take longer than in a young child. All other previous cases have been in children. It is also possible that this is a related virus and not the usual putative agent of HFAM Disease.

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Retroauricular Dermatitis

Abstract: 16 yo boy with 3-4 year history of retroauricular dermatitis
History: This 16-year-old boy was seen for evaluation of a retroauricular dermatitis that has been present for 3-4 years. He is in his usual state of health. He does not have a history of atopy. He does not wear glasses.
O/E: Honey-colored crusting in the superior retroauricular sulci bilaterally.
Clinical Photo:

click image to enlarge
Lab: Culture positive for many Staph. aureus with usual sensitivities.
Histopathology: N/A
Diagnosis or DDx: Retroauricular Dermatitis: This is felt to be a marker for atopic dermatitis or atopy. However, this boy is not atopic and the finding may not be all that specific. There is only one article has appeared on this subject (see Reference).
Treatment: The patient was given a sample tube of retapamulin ointment (Altabax) to use b.i.d. for one week. The next photo shows appearance after one week of use as monotherapy. I plan to now use fluocinalone 0.025% ointment daily for a week or two for the residual dermatitis. This may well recur. The natural history of retroauricular dermatitis is poorly defined. There is only one article in the medical literature that discusses this entity.

status post 0ne week of retapamulin ointment

Questions: Does anyone have any comments on this entity? How often do you see this? I see one or two cases a year.
Reason(s) Presented: For interest. It is curious that there are no more reports on this since it appears to be an entity.
References:
Marks MB, et. al. An unsuspected sign of cutaneous allergy. J Am Acad Dermatol. 1981 May;4(5):519-22.

An eczematous eruption in the superior retroauricular areas of the scalp and often
on the posterior aspects of the pinnas may be seen in about 30% of allergic
children. The eruption is not generally noticed because the overhanging hair covers
the affected areas. The dermatitis is seen mainly in those children afflicted with
bronchial asthma, perennial allergic rhinitis, or both. A previous history of atopic
or seborrheic dermatitis is, as a rule, not elicited.

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Scalp Folliculitis in a Patient on Chemotherapy

HPI: This 55 yo woman has had a folliculitis of her scalp for the past 2 - 3 weeks. She is receiving taxol and carboplatinum every three weeks for ovarian cancer and has had two infusions thus far. This eruption began after the second infusion.

O/E: Alopecia secondary to chemotherapy. Scattered over the scalp are erythematous papules and pustules. There are no other lesions other than on the scalp.

Clinical Photos:




Lab: Bacterial culture obtained.

Pathology: Can consider biopsy

Diagnosis: Folliculitis. Probably related to Taxol.

Discussion: A Medline search found one reference to Taxol and folliculitis. This was a case report of two men with folliculitis of the bearded areas and chests after Taxol infusions. Folliculitis is also reported in women on Taxol, but there is no literature available on the subject.

Reason Presented: I discussed her findings with her oncologist who said he sees this picture frequently. It's peculair that there are no case reports. Folliculitis can be bacterial, sterile, fungal or even eosinophilic pustular folliculitis. A biopsy might help. In the absence of guidelines, I started the patient on doxycycline 100 mg. bid. If anyone has seen and treated a similar patient, I would appreciate your insights and recommendations.

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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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