Transient myeloproliferative disorder–associated cutaneous pustulosis

P3001

P3003

Transient myeloproliferative disordereassociated cutaneous pustulosis John Bernardi, MD, University of Louisville, Louisville, KY, United States; Jeffrey Callen, MD, University of Louisville, Louisville, KY, United States; Soon Bahrami, MD, University of Louisville, Louisville, KY, United States Transient myeloproliferative disorder (TMD) is a self-limited leukocytosis that affects 10% of neonates born with trisomy 21 (Down syndrome). Acquired mutations in GATA-1 have been implicated in the pathogenesis of TMD. Because of this GATA1 mutation, TMD is sensitive to the therapeutic effects of cytarabine (Ara-C). Despite its usual benign nature, patients with a history of TMD should be periodically evaluated for evidence of leukemic relapse. Although TMD is well-known, only rarely has it been described in association with a coinciding pustular eruption which mirrors the hematologic abnormalities. A Hispanic boy with clinical manifestations of Down syndrome was born with a leukocytosis of 498,000 consisting of 63% blasts. TMD was confirmed with GATA-1 mutation analysis. On day 1 of life, occasional scattered pustules arose on the scalp and face. Viral, fungal, and bacterial cultures from lesional skin were negative. Direct fluorescent antibody testing was negative for herpes simplex and varicella zoster virus. Cerebrospinal fluid herpes viral evaluation was also negative. The patient remained afebrile and hemodynamically stable in an intensive care setting. Dermatology was consulted on day 4 of life for progression of disease. On physical examination, numerous grouped pustules on an erythematous base were noted most prominently in a perioral and periocular distribution. Pustules of the scalp, chest, and abdomen were also appreciated on skin previously traumatized by adhesive tape. A 3-mm punch biopsy specimen was obtained from the right upper chest. Histopathologic evaluation revealed an atypical hematolymphoid infiltrate exhibiting marked exocytosis and subcorneal vesiculation. CD33 and myeloperoxidase immunopositivity confirmed the myeloid lineage of the atypical cells. These findings supported our clinical suspicion of TMD associated cutaneous pustulosis. Hematology/oncology initiated therapy with cytarabine. A resolution of the leukocytosis was seen over 3 days, along with resolution of his cutaneous findings. No new pustules were appreciated during the hospital stay. This case highlights the parallel courses followed by TMD and its cutaneous pustulosis culminating in complete resolution with appropriate therapy. Strong consideration should be given to this diagnosis in any neonate with a leukocytosis and predominant facial vesico-pustules. Phenotypically normal neonates, mosaic for trisomy 21, are also at risk.

Kwashiorkor attributed to rice milk in a child with atopic dermatitis and food allergies Julie Fenner, MD, University of Massachusetts, Worcester, MA, United States; David Miller, University of Massachusetts, Worcester, MA, United States; Leah Belazarian, MD, University of Massachusetts, Worcester, MA, United States; Rujing Han, MD, University of Massachusetts, Worcester, MA, United States; Vanessa Laffert, University of Massachusetts, Worcester, MA, United States We report a case of a 13-month-old female with a history of moderate atopic dermatitis and food allergies who presented with a 4-week history of anasarca and flaky paint dermatosis. She was irritable and lethargic and had diffuse nonpitting edema, most prominent on the eyelids, nose, lips, oral mucosa, and legs. There were well-demarcated pink plaques with superficial red-brown scaling on the right cheek, chest, thighs, and lower legs. Her abdomen was protuberant. Her nails and hair appeared normal. Because of multiple food allergies, including to milk, her source of dairy had been changed to rice milk and her diet lacked other sources of protein. A punch biopsy specimen of skin of the left thigh showed mild epidermal hyperplasia with compact parakeratosis, pallor of the upper epidermis with focal vacuolar change, and a perivascular lymphoid infiltrate in the superficial dermis, findings that are consistent with a nutritional deficiency. Laboratory work-up revealed a low total protein (3.3 g/dL), albumin (1.1 g/dL), and prealbumin (11 mg/dL). She was diagnosed with kwashiorkor, which resolved promptly with protein supplementation. While the incidence of childhood malnutrition is estimated to be greater than 20% globally, only 1% of children in developed countries are afflicted by undernutrition. Findings typically associated with kwashiorkor include edema, low albumin, and a dermatosis. Skin findings are characterized by red-brown plaques with superficial scaling that lifts at the edges, producing what has been called ‘‘flaking paint dermatosis.’’ Hypopigmentation of the hair root can cause bands of light and dark discoloration of the hair, producing a finding often referred to as the ‘‘flag sign,’’ which our patient eventually developed. Because of the limited incidence of kwashiorkor in developed countries, the index of suspicion of this life-threatening condition is very low. Allergy avoidance as a cause for kwashiorkor has been reported in the United States. Children who have an allergy to milk may be started on replacement diets that are deficient in protein. In this case, rice milk was the child’s milk source, which is not an adequate source of protein. Awareness of the cutaneous manifestations may lead to prompt diagnosis and early intervention. Patients should be educated about nutrition when avoidance diets are initiated because of food allergies.

Commercial support: None identified.

Commercial support: None identified.

P3002 Investigation of recreational, acute solar UVR exposure: Effects on skin of Hispanic and white children M. Catherine Mack, Johnson and Johnson Consumer Companies, Skillman, NJ, United States; Georgios N. Stamatas, PhD, Johnson and Johnson Consumer France, Issy-les-Moulineaux, France; Katharine M. Martin, Johnson and Johnson Consumer Companies, Skillman, NJ, United States; Nikiforos Kollias, PhD, Johnson and Johnson Consumer Companies, Skillman, NJ, United States Background: Previous studies have shown that infant skin differs from adult skin and continues to develop over the first years of life. Recently, we revealed that facultative pigmentation caused by incidental solar ultraviolet radiation (UVR) is detectable as early as the first summer of life in white infants. To further our understanding of the effects of sun exposure in young children, we evaluated the changes in skin properties of children aged 2 to 5 years during acute, recreational solar UVR exposure, such as that experienced after a day at the beach. Hispanic and white children were compared in order to determine whether skin responses differ based on complexion (ie, constitutive pigmentation levels). Methods: Nineteen Hispanic and 22 white children were enrolled, with mothers included for comparative purposes. Subjects were instructed to follow their normal routine during a vacation day at the beach. Sunscreen (SPF 50) was provided. Subjects were evaluated the morning before the beach day, the evening of the beach day, the morning of the next day (z24 hours after sun exposure), and 1 week after the beach day. At all time points, digital images were taken of the face, and measurements were taken on the dorsal forearm and shoulder (skin color, chromophore apparent concentrations). On the dorsal forearm and shoulder, an opaque adhesive bandage was placed on the skin next to the measurement site for the duration of the day spent at the beach to provide a control site unexposed to the sun. Results: Hispanic children (but not Hispanic adults) displayed persistent increases in melanin concentration after the beach day, indicating that a single day of recreational solar UVR exposure is sufficient to induce de novo synthesis of melanin in Hispanic children. While levels of melanin were similar between Hispanic children and adults at baseline, the skin’s response to sun exposure was different between children and adults. Facial erythema levels increased in Caucasian adults after a day at the beach, but significant changes in erythema levels in white children were not observed. Conclusion: These results show that constitutive pigmentation does not predict induced melanin production. An individual’s response to sun exposure cannot be predicted by their complexion. This highlights the need for sun protection in all children, including those of medium to dark complexion. Commercial support: 100% sponsored by Johnson and Johnson Consumer Companies.

FEBRUARY 2011

P3004 What is different in skin physiology in neonates and young children of different age groups compared to adults? A randomized in vivo study Joachim Fluhr, Bioskin, Hamburg, Germany; Clarence de Belilovsky, Institut Alfred Fournier, Paris, France; Nadege Lachmann, Laboratoires Expanscience, Epernon, France; Philippe Msika, Laboratoires Expanscience, Epernon, France Background: The skin of neonates and children has anatomic and physiologic differences to adults, including water content and perspiration, light sensibility, percutaneous permeability, susceptibility to infections and irritants, and topical treatments. The aim of the present study was to investigate noninvasively physiologic skin parameters (transepidermal water loss [TEWL], stratum corneum [SC] hydration, and surface pH) and the biochemical skin composition (water profile and bulk NMF) to characterize neonatal skin in comparison to different children age groups and adults skin. Methods: The study was performed in healthy male and female volunteers (n ¼ 108) of six age groups (n ¼ 18 each group: newborns [1-15 days], 5-week, 6-month, 1-2 years, 4-5 years, and adults aged 20-35 years). Biophysical parameters (TEWL, capacitance, and surface pH) were measured on the volar forearms. In six subjects of each age groupm water and bulk NMF profiles where assessed with in vivo Raman spectroscopy (RS). Results: The lowest SC hydration was noted in newborns (1-15 days) compared to other age groups, representing a dry SC in newborns. The mean skin surface pH value was highest in newborns. A decrease of approximately 1 pH unit was observed over the first 5 weeks and comparable to lower pH values in all other age groups, representing a quick stabilization of pH within the first weeks of life. In the present study under basal conditions all mean TEWL values were below 10 g/m2/h, reflecting a normal and competent barrier function in all six age groups. An increase in SC hydration within the first weeks of life was detected in the water profile assessed with RS measurement. The newborns showed a slower increase in water content in correlation to increasing skin depth compared to the other age groups. Newborns showed greater bulk NMF concentrations—especially in depths of 5 to 15 m. It seems that the lack of water in mid and deeper layers is compensated by a high NMF level in the skin surface induced by filaggrin breakdown after birth. Conclusion: Neonatal and infant skin is mainly competent under basal conditions except SC water content and NMF components regulating the SC hydration level. Newborns are adapting to the dry environment by compensating the lack of water with increased NMF. Commercial support: None identified.

J AM ACAD DERMATOL

AB129