Wednesday, February 27, 2013

Dermatoscopy of Molluscum

The patient, a two year old girl, was referred for evaluation of a 5 mm in diameter tumor on the left shoulder present for a few months.  The lesion was a dome-shaped papule that was in the presence of numerous smaller but similar lesions (the latter were typical molluscum).

Dermoscopy of molluscum has been described in the literature.  The most salient feature is presence of polylobular amorphous white to yellowish globules as seen in the larger of the two lesions below.  The smaller lesion shows just a solitary amorphous lobule.

Discussion:  Dermatoscopy is a useful tool for the diagnosis of molluscum.  The presence of the white globules (which represent the molluscum bodies) is a pathognomonic sign.  In this large lesion, multiple molluscum bodies are the tip-off to the diagnosis.

Cliff Rosendahl writes: “This is a non-pigmented lesion, circular in shape with a sharply demarcated border over the total periphery. Centrally there are white clods and white structureless areas on a pink background with serpentine vessels.  In ‘Dermatoscopy’ page 236 the characterisation is “White to yellow clods or structureless zone and curved vessels at the periphery which do not cross the centre” so this example is a variation of that description.”

1. An Bras Dermatol. 2011 Jan-Feb;86(1):74-9.
Dermoscopic patterns of molluscum contagiosum: a study of 211 lesions confirmed by histopathology.  Free full text
RESULTS: At clinical examination and dermoscopy of 211 lesions, orifices were visualized in 50.24% and 96.68% of the lesions, and vessels in 6.16% and 89.10%, respectively. The vascular patterns found in the 188 lesions in which vessels were found at dermoscopy were the crown (72.34%), radial (54.25%) and punctiform patterns (20.21%). Half of the 188 lesions had a combination of vascular patterns, with the flower pattern (a new vascular pattern) being found in 19.68% of cases. More orifices and vessels were identified at dermoscopy than at clinical examination, including cases with inflammation or perilesional eczema and small lesions. Punctiform vessels were associated with inflammation, excoriation and perilesional eczema.
CONCLUSIONS: Dermoscopy performed on molluscum contagiosum lesions proved superior to dermatological examination even in cases in which clinical diagnosis was difficult. The presence of orifices, vessels and specific vascular patterns aids diagnosis, including differential diagnosis with other types of skin lesion.  

2.  Arch Dermatol. 2005 Dec;141(12):1644.  Dermoscopy of molluscum contagiosum.
Morales A, Puig S, Malvehy J, Zaballos P.

Wednesday, February 20, 2013

Transverse Leukonychia

Dr. Richard Ratzan, an Emergency Room physician in Connecticut, recently saw an institutionalized bipolar woman in her 50s for another problem and noted a distinctive nail dystrophy.  It looks like transverse leukonychia of which there are only a few references in the literature.  The woman has a history of biting but no significant medical history that he is aware of.  We don't know if she's had electroshock therapy or intermittent chemotherapy that may have been contributory.  Could some type of self-induced trauma have caused this?

Dr. Ratzan writes us: "Although i am not a dermatologist, i have long been interested in physical diagnosis and especially nails. i usually take a good look at my patients' nails. What struck me as interesting in this man was the following: i had never seen this pattern before; i could not and still can not imagine a pathophysiological process leading to such an unusual symmetrical pattern of feathered chevrons that were non-continuous across the longitudinal midline of the nail; and lastly, from a strictly aesthetic point of view, i find them almost weirdly beautiful!"

Discussion:  Transverse Leukonychia have been reported a number of times in the medical literature but most cases have been associated with combined chemotherapy or some other pharmacologic agents.  Reference # 3 is interesting but there is no abstract and the source is difficult to find.  Does anyone have access to this?  Muehrcke Lines and Mee's lines would appear to be different entities often confused with this type of transverse leukonychia.  Or, perhaps we are missing something inthis woman's history.

1. Arch Dermatol. 1983 Apr;119(4):334-5.  Chemotherapy-induced transverse white lines in the fingernails.  James WD, Odom RB.  (A good review, but no abstract – only useful for those with access to Archive of Derm)
2.  CMAJ. 2012 Aug 27.  Muehrcke lines  Sharma V, Kumar V. (Shows why our patient does not have this.)
3.  Traumatic transverse leukonychia.  Maino KL, Stashower ME.  Skinmed. 2004 Jan-Feb;3(1):53-5. No abstract available.

Wednesday, February 13, 2013

Newborn with Erosive Diaper Dermatits

Abstract:  5 week old girl with an erosive diaper dermatitis since day 2.
HPI:  The patient was born at 3200 grams to a P2G2 opioid-addicted mother who was on Suboxone (buprenorphine and naloxone) maintenance.  The infant is feeding well and gaining weight and the mother seems attentive.  At day 2 her mother noted the process seen below.  The child is bottle fed and had originally been on Enfamil but was switched to Nutramagen.  Treatment to date has been with a panoply of creams:  Neomycin (!), Desitin, clotrimazole.  The mother relates that stools seem unremarkable.

O/E:  There is a nodulo-ulcerative napkin dermatitis.  No other skin lesions are noted.

Clinical Photo:

Lab:  A culture was done and was negative.  KOH not done.

Diagnosis:  Jacquet-type Nodulo-ulcerative Diaper Dermatitis in an infant with possible narcotic abstinence syndrome (see reference).

How many of you have seen a similar case of Jacquet’s Disease in an neonate?  What local care would you recommend?  Any work-up at this point?  Are you aware of the narcotic abstinence syndrome and if so have you seen an erosive napkin dermatitis like this?

My approach at this time is to try the alpha tocopherol, keep diaper changes simple, consider cloth diapers for 1 – 2 weeks.  KOH prep will be done.  If response is not favorable consider biopsy or admission to hospital for care.

One Month Follow-up:
The baby was treated in the following way, as recommended by Dr. Julianne Mann from Pediatric Dermatology, OHSU, Portland, Oregon:

1. Absolutely NO diaper wipes at all.  Have mom get a large pack of rectangular cotton makeup remover pads.  Have her apply mineral oil to the cotton pad and use this to clean the baby's bottom.  When she is away from home OK to have her use a damp cotton cloth (I tell parents to pack several damp soft cotton washcloths in a ziplock in their travel diaper bag).

2. With every diaper change, have mom liberally apply a thick barrier paste after cleaning the baby's bottom.  We have had the best luck with a compounded butt paste that our local pharmacy mixes up because we suspect that preservatives in OTC diaper pastes may also play a role with the dermatitis.  We do 25% corn starch, 25% zinc oxide, and 50% petrolatum and dispense one pound.

3. I emphasize the importance of stopping everything else that they've been using.  Most parents whose baby's butt looks like this have been desperately trying everything, including doing things like vinegar or baking soda soaks and trying a different cream almost daily.  I tell them the goal is to simplify.  KEEP IT SUPER SIMPLE!
Thank you, Lili!!


[Effectiveness of topical acetate tocopherol for the prevention and treatment of skin lesions in newborns: a 5 years experience in a 3rd level Italian Neonatal Intensive Care Unit].  Manzoni P, Gomirato G.  Minerva Pediatr. 2005 Oct;57(5):305-11.
Divisione di Neonatologia e TIN Ospedaliera, Ospedale S. Anna, Azienda Ospedale OIRM-S. Anna, Turin.
Abstract:  Neonates in NICU (especially when premature) are particularly prone to skin damage by action of external aggressive conditions such as chemical, physical, infectious, radiant, mechanical and iatrogenic factors. Strategies for avoiding disruption of the skin barrier are thus highly needed in such patients.
METHODS: We evaluated the effectiveness of a acetate tocopherol (AT) ointment for topical use in 21 neonates admitted to our NICU and affected by neonatal abstinence syndrome with severe diaper exulcerative and erosive erythema with ulcer and granulation tissue at the bottom of the lesion (group A), and compared them to 19 matched neonates affected by the same condition and treated with a commonly used skin ointment (emollient type, water-in-oil category) (group B). For all newborns we calculated: the dermatological severity score (using a clinical score from 0 to 9 points according to the increasing severity of the lesions) at time 0, 4 and 7 days; the mean days for achieving complete recovery; the rate of therapeutical failures.
RESULTS:  Mean score at day 0 was 7.8 in group A vs 7.9 in group B (P=0.35 NS). At day 4 it was 4.6 in group A vs 6.5 in group B (P=0.03), at day in 7 it was 3.1 in group A vs 5.2 in group B (P=0.04). A complete recovery with restitutio ad integrum occurred after 9.1 mean days in group A vs 12.2 mean days in group B (P=0.04). The rate of therapeutical failures was significantly lower in group A (4.2% vs 30.6%; OR 0.235; P<0.01) than in group B. No adverse effects related to AT use were reported.
CONCLUSIONS:  AT in our experience proved to be safe and more effective than the commonly used skin ointments in the topical treatment of exulcerative skin lesions in NICU neonates.

Wednesday, February 06, 2013

Porphyria Cutanea Tarda

Abstract:  50 yo man with erosions on dorsum of hands, arms, elbows, and scalp for six months.

HPI: This 50 year-old man reports he has had "blisters and sores" on dorsum of hands, arms, elbows, and scalp for six months.  He feels he is in otherwise good health, takes no meds by mouth and drinks 2 - 3 beers per day.  This man works outdoors as a carpenter so he gets lots of sun (although it is winter here). When he developed his initial lesions it was summertime.

O/E:  There are superficial erosions of the hands, elbows, and scalp.  No vesicles were noted.

Clinical Photos:

Lab: CBC normal
Chemistries:  SGOT 141 (normal 10 - 42)
SGPT  164  (normal 19 - 49)
Ferritin 486  (normal  220 - 250
Urinary Porphyrins:
Uroporphyrin:  1523 (normal < 22)
Coprophyrin: 450 (23 - 230)
Total Porphyrins  2675  (normal  31 - 139)
Hepatitis Serology and HIV tests willbe ordered.

Diagnosis:  Porphyria Cutanea Tarda

Plan:  Will probably treat with hydroxychloroquine



1. Clin Gastroenterol Hepatol. 2012 Dec;10(12):1402-9
Low-dose hydroxychloroquine is as effective as phlebotomy in treatment of patients with porphyria cutanea tarda.
Singal AK, Kormos-Hallberg C, Lee C, Sadagoparamanujam VM, Grady JJ, Freeman DH Jr, Anderson KE.
Department of Preventive Medicine and Community Health, University of Texas Medical Branch, Galveston, TX, USA.
BACKGROUND:  Porphyria cutanea tarda (PCT) is an iron-related disorder caused by reduced activity of hepatic uroporphyrinogen decarboxylase; it can be treated by phlebotomy or low doses of hydroxychloroquine. We performed a prospective pilot study to compare the efficacy and safety of these therapies.
METHODS: We analyzed data from 48 consecutive patients with well-documented PCT to characterize susceptibility factors; patients were treated with phlebotomy (450 mL, every 2 weeks until they had serum ferritin levels of 20 ng/mL) or low-dose hydroxychloroquine (100 mg orally, twice weekly, until at least 1 month after they had normal plasma levels of porphyrin). We compared the time required to achieve a normal plasma porphyrin concentration (remission, the primary outcome) for 17 patients treated with phlebotomy and 13 treated with hydroxychloroquine.
RESULTS: The time to remission was a median 6.9 months for patients who received phlebotomy and 6.1 months for patients treated with hydroxychloroquine treatment (6.7 and 6.5 mo for randomized patients), a difference that was not significant (log-rank, P = .06 and P = .95, respectively). The sample size was insufficient to confirm noninferiority 

2. Liver Int. 2012 Jul;32(6):880-93
Hepatitis C, porphyria cutanea tarda and liver iron: an update.
Ryan Caballes F, Sendi H, Bonkovsky HL
The Liver-Biliary-Pancreatic Center of Carolinas Medical Center, Charlotte, NC, USA.
Abstract: Porphyria cutanea tarda (PCT) is the most common form of porphyria across the world. Unlike other forms of porphyria, which are inborn errors of metabolism, PCT is usually an acquired liver disease caused by exogenous factors, chief among which are excess alcohol intake, iron overload, chronic hepatitis C, oestrogen therapy and cigarette smoking. The pathogenesis of PCT is complex and varied, but hereditary or acquired factors that lead to hepatic iron loading and increased oxidative stress are of central importance. Iron loading is usually only mild or moderate in degree [less than that associated with full-blown haemochromatosis (HFE)] and is usually acquired and/or mutations in HFE. Among acquired factors are excessive alcohol intake and chronic hepatitis C infection, which, like mutations in HFE, decrease hepcidin production by hepatocytes. The decrease in hepcidin leads to increased iron absorption from the gut. In the liver, iron loading and increased oxidative stress leads to the formation of non-porphyrin inhibitor(s) of uroporphyrinogen decarboxylase and to oxidation of porphyrinogens to porphyrins. The treatment of choice of active PCT is iron reduction by phlebotomy and maintenance of a mildly iron-reduced state without anaemia. Low-dose antimalarials (cinchona alkaloids) are also useful as additional therapy or as alternative therapy for active PCT in those without haemochromatosis or chronic hepatitis C. In this review, we provide an update of PCT with special emphasis upon the important role often played by the hepatitis C virus.