Gluteal Abscess

Abstract: 94 yo man with gluteal abscess.

HPI:  The patient is a 94 yo man in fair general health.  Hi has chronic pruritus of "Willan's Itch" and has been on prednisone 10 mg daily for a year to control his intractable pruritus.  He sits for hours per day because of a tendon tear around the left knee that severely restricts ambulation.  His mental status is fair.  He has no marked dementia, but has slowed down in the past couple of years.  Other than orthopedic problems he has no serious medical disorders.

O/E:  Red, tender mass in the left buttock.

Photo: 

Lab:  Culture taken from crusted area grew moderate Saph aureus sensitive to everything except penicillin

Procedure:  The patient was sent to a surgeon who did and I and D and packed the area.  He reported ~ 40 cc of pus admixed with blood.

At present the patient is home, fairly comfortable, on cephalexin 500 mg tid.  Packing is changed daily.

Diagnosis:  Gluteal abscess.

Questions:  This is a somewhat frail 94 yo man.  He does not want to enter hospital.  I am concerned that there may be loculations that have not been probed.  Considering the organism, is cephalexin enough? 

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Whose Nose Needs Mohs?

Abstract:  77 yo woman with 5 mo history of pigmented macule on nose

HPI:  77 yo woman in fair general health with 5 mo history of evolving lesion on bridge of nose.

O/E: 1.5 x1.0 macule with slight play of color bridge nose.

Clinical photos: Arrows show original biopsy sites.

Biopsies:
1) 2 x 3 mm punch bx taken from areas indicated by arrows.  "Mild to moderate atypical melanaocytic hyperplasia with focal pagetoid spread.
2) Incisional biopsy of most (not all) of residual lesion. "Lentiginous melanocytic hyperplasia best interpreted as 'melanoma in situ.' In the appropriate clinical setting complete reexcision is recommended for further evaluation and management."

(Biopsies were signed out by two different dermatopathologists at the same facility.)

Photomicrographs courtesy of Dr. Deon Wolpowitz, Boston University, Department of Skin Pathology.

Mart 1 Positive 

Diagnosis:  Atypical Melanocytic Hyperplasia vs. Melanoma in situ

Discussion: Clearly, a more complete excision would be problematic.  This lesion would appear to have minimal potential to metastasize and the plan here would be either wait and watch or imiquimod.  What would your approach be? 
What is highlighted here is the subtle change of diagnosis from atypical melanocytic hyperplasia to M.I.S.  Semantics certainly makes a difference.

Questions: Would you refer to a Mohs surgeon or  treat with imiquimod?  What is the risk for invasion in such a lesion?

About MART 1 Stain: MART-1 has nothing to do with prognosis or treatment.  It is basically a staining tool to identify melanoma cells in a tissue sample.  Having MART-1 positive means melanocytes showed up on the sample and the pathology can confirm melanoma.  MART-1 is specific to melanoma so when cells stain positive, there are melanocytes there.  It also shows normal melanocytes, but if it stains positive in a tissue sample that shouldn't contain melanocytes (tumors), then you have a key to diagnosis

References:

1.  An Bras Dermatol. 2011 Jul-Aug;86(4):792-4.

Lentigo maligna treated with topical imiquimod: dermatoscopy usefulness in clinical monitoring.

[Article in English, Portuguese]  Free Open Access

Costa MC, Abraham LS, Barcaui C.

Instituto de Dermatologia Prof. Rubem David Azulay, Santa Casa da Misericórdia do Rio de Janeiro, Brasil.

Abstract: Dermoscopy has its usefulness well established in the diagnostic evaluation of melanocytic lesions. Recently, however, it has also shown to be an important tool in monitoring therapeutic response to various dermatoses. We report the case of an elderly patient diagnosed with lentigo maligna of difficult surgical management, which we have chosen to treat with topical imiquimod. The dermoscopic monitoring of this alternative therapy has shown to be of great usefulness.

2.  Case Rep Dermatol. 2009 Oct 31;1(1):78-81.

Topical Imiquimod Treatment of Lentigo Maligna.

Ventura F, Rocha J, Fernandes JC, Pardal F, Brito C.

Source: Department of Dermatology and Venereology, Hospital de São Marcos, Braga, Portugal. Free Open Access

Abstract: Lentigo maligna (LM) is the in situ phase of lentigo maligna melanoma, which may progress to invasive melanoma if left untreated. It mainly occurs on sun-exposed areas of elderly patients. The lesions can be large and conventional surgery can be difficult, particularly on the face. Recent reports indicate that topical imiquimod 5% cream is effective in the treatment of LM. It may be an alternative when surgery or other classical treatments are not possible in elderly patients. We describe an 80-year-old Caucasian woman with a 10-year history of a histologically verified extensive LM of the face. She was treated with imiquimod 5% cream once daily. After four months it showed complete clinical response. One year after the treatment the patient was still free from recurrence.as shown to be of great usefulness.

3. 

--> Clin Med Oncol. 2008; 2: 551–554.

Observational Study of Topical Imiquimod Immunotherapy in the Treatment of Difficult Lentigo Maligna

E.E. Craythorne and C.M. Lawrence  Free Open Access.

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Scald-like Eruption in a Newborn

Dr. Yogesh Jain from Ganiyari, Bilaspur  in Chhattisgarh, central India requests your help with this infant.

The first two pics are of a 22 day old newborn girl who started developing erythematous lesions on the face, on arms and the abdomen at the age of 15 days and now has it for 7 days. After initial erythema, it darkens and becomes scald-like, as seen below. No blisters . No nail involvement . No feeding problem. No problem with hair. Sometimes these lesions get a little bit of pus, but not at this moment. The parents are non-consanguineous.

Her elder sib, a girl, also developed this at the age of 15 days, mainly on the face. Now at 7 years, she has hyperpigmented lesions on the face. Otherwise normal.

A first cousin also had similar lesions.

What is this?

Clinical Images of proband, her sister and cousin.

Heals without ectropion

Diagnosis:  This may be a unique variant of Self-Healing Collodion Baby with limited expression.

Discussion:  It's hard to explain this as anything other than an autosomal recessive disorder since none of the parents are affected (need to confirm by history).  Reference 3. is available as a full text.  I would imagine that cool compresses with clean water and an emollient with a petrolatum base for a few weeks may speed and aid healing.  We will try to get some opinions from pediatric dermatologists.

References:
1. Autosomal Recessive Congenital Ichthyosis.

[Article in English, Spanish]

Rodríguez-Pazos L, Ginarte M, Vega A, Toribio J.

Actas Dermosifiliogr. 2012 Jul 13. [Epub ahead of print]

Departamento de Dermatología, Complejo Hospitalario Universitario, Facultad de Medicina, Santiago de Compostela, España.

Abstract: The term autosomal recessive congenital ichthyosis (ARCI) refers to a group of rare disorders of keratinization classified as nonsyndromic forms of ichthyosis. This group was traditionally divided into lamellar ichthyosis (LI) and congenital ichthyosiform erythroderma (CIE) but today it also includes harlequin ichthyosis, self-healing collodion baby, acral self-healing collodion baby, and bathing suit ichthyosis. The combined prevalence of LI and CIE has been estimated at 1 case per 138 000 to 300 000 population. In some countries or regions, such as Norway and the coast of Galicia, the prevalence may be higher due to founder effects. ARCI is genetically highly heterogeneous and has been associated with 6 genes to date: TGM1, ALOXE3, ALOX12B, NIPAL4, CYP4F22, and ABCA12. In this article, we review the current knowledge on ARCI, with a focus on clinical, histological, ultrastructural, genetic, molecular, and treatment-related aspects.

2. Acral self-healing collodion baby: report of a new clinical phenotype caused by a novel TGM1 mutation.Mazereeuw-Hautier J, Aufenvenne K, Deraison C, Ahvazi B, Oji V, Traupe H, Hovnanian A.  Br J Dermatol. 2009 Aug;161(2):456-63.

Department of Dermatology, CHU Purpan, Toulouse 31024, France. mazereeuw-hautier.j@chu-toulouse.fr

Abstract:  A minority of collodion babies, called 'self-healing collodion babies', heal spontaneously. We describe a novel clinical phenotype of acral self-healing collodion baby caused by a new TGM1 mutation. The proband, born to healthy parents, presented at birth as a collodion baby strictly localized to the extremities. The skin condition returned to normal at the age of 3 weeks. The older sister was born as a generalized collodion baby; the condition then developed into lamellar ichthyosis. Molecular analysis of TGM1 revealed three novel mutations in the family. The proband was compound heterozygous for the p.Val359Met and p.Arg396His mutations, whereas the older sister was compound heterozygous for p.Arg396His and a deletion mutation c.1922_1926+2delGGCCTGT. Structural modelling of the p.Val359Met mutation suggested a minor disruption of the protein structure, whereas a modification of protein-protein interaction was predicted for p.Arg396His. These predictions corroborated the analysis of recombinant transglutaminase (TGase)-1 proteins carrying the p.Val359Met and p.Arg396His mutations. Both showed decreased levels of protein expression: p.Val359Met displayed residual activity (12.8%), while p.Arg396His caused a dramatic loss of activity (3.3%). These observations demonstrate for the first time that TGM1 mutations can be associated with acral self-healing collodion baby, and expand the clinical spectrum of TGase-1 deficiency.

2. Genotypic and clinical spectrum of self-improving collodion ichthyosis: ALOX12B, ALOXE3, and TGM1 mutations in Scandinavian patients. Full Text

 J Invest Dermatol. 2010 Feb;130(2):438-43. Vahlquist A, et. al.

Source

Department of Medical Sciences, Uppsala University, Uppsala, Sweden.

Abstract: Infants born with autosomal recessive congenital ichthyosis (ARCI) are often encapsulated in a collodion membrane, which shows a lamellar or erythrodermic type of ichthyosis upon shedding. However, some babies show a nearly normal underlying skin after several weeks, a phenotype called "self-healing collodion baby" (SHCB). Mutations in two genes, TGM1 and ALOX12B, have previously been implicated in the etiology of SHCB, but the full genotypic spectrum remains to be determined. DNA sequencing in 11 Swedish and 4 Danish SHCB patients showed ALOX12B mutations in eight cases, ALOXE3 mutations in three cases, and TGM1 mutations in one case. In three patients, we could not find mutations in any of the known ARCI genes. In all cases, a spontaneous shedding of the collodion membrane occurred 2-4 weeks after birth. When re-examined at 2-37 years of age, the patients showed skin xerosis, a mild or focal scaling, palmar hyperlinearity with keratoderma, and a frequent appearance of red cheeks and anhidrosis. Thus, we propose replacing SHCB with the term "self-improving collodion ichthyosis" (SICI). In conclusion, ALOX12B mutations are the leading cause of SICI in Scandinavia, followed by ALOXE3 mutations, which have not been previously associated with this variant of ARCI.

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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.”

References:

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

Abstract

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.

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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.

References:

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.

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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).

Questions:

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!!

Reference:

[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. paolomanzoni@hotmail.com

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.

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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

Questions: 

Reference:

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. aksingal@uab.edu

Abstract

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.

 

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