Solitary cutaneous infantile myofibroma as a hallmark of myofibromatosis: Two cases and review of the literature.

Infantile myofibroma (IM) commonly presents as a benign cutaneous fibrous tumor in infancy. Although the majority of solitary IM regress without any morbidity, some cases have underlying bone or visceral involvement that can lead to both morbidity and mortality. In this report with review of the literature, we present two cases of solitary cutaneous IM with internal involvement and discuss screening cases of solitary IM with full body imaging.

A case of Henoch-Schonlein Purpura with dilated coronary arteries.

BACKGROUND: Henoch-Schonlein Purpura (HSP) is one of the most common vasculitides of childhood, with 10-20 cases per 100,000 children. It frequently occurs following an infectious trigger and involves IgA and C3 deposition in small vessel walls. HSP is characterized by palpable purpura plus IgA deposition on biopsy, arthritis/arthralgia, renal involvement (hematuria and/or proteinuria), and/or abdominal pain. It is not generally recognized as a cause of dilated coronary arteries. CASE PRESENTATION: We describe the first reported case of HSP presenting with dilated coronary arteries. This patient is a nine-year-old previously healthy Caucasian male who presented with 1 week of petechiae on his lower legs, knee and ankle arthritis, and abdominal pain without fever, consistent with HSP. An echocardiogram revealed coronary dilation, including the left main (5.32 mm, Z score + 4.25) and left anterior descending (LAD) (3.51 mm, Z score + 2.64) coronary arteries. He received high dose aspirin, IVIG, and infliximab with normalization of the LAD. Skin biopsy revealed leukocytoclastic vasculitis with positive IgA staining. He was Rhinovirus/Enterovirus positive with Group A Streptococcus on throat culture. CONCLUSION: Cardiac findings, while rare, can exist in HSP. Coronary dilation appeared to respond to our hospital protocol's Kawasaki Disease (KD) therapy, possibly indicating an overlap in HSP and KD pathophysiology. This case, along with prior reports of dilated coronaries in systemic juvenile idiopathic arthritis (SJIA), highlights the importance of considering other sources of systemic inflammation, in addition to KD, when coronary dilation is identified. The appropriate therapy, follow-up, and prognosis for our patient are not clear, as further studies are needed to determine the natural course of these findings.

Index Case of Cutaneous Follicular Mycosis Fungoides With Central Nervous System Involvement and Review of Literature.

Central nervous system involvement by mycosis fungoides (MF) is rare and is usually seen in advanced stages of the disease. We describe a patient with early-stage follicular MF who presented with changes in mental status. Despite an initial diagnosis of vasculitis based on clinical and brain biopsy results, the postmortem examination revealed extensive infiltration of MF cells throughout the brain with leptomeningeal involvement. This case in addition to the accompanied review of literature illustrates the importance of the awareness of central nervous system involvement by MF and highlights the need for an urgent neurologic evaluation in patients with a history of MF now presenting with neurologic signs or symptoms.

Characterization of Children With Recurrent Episodes of Stevens Johnson Syndrome.

We performed a retrospective chart review for all cases of recurrent Stevens Johnson Syndrome (SJS) from March 2013 to March 2016. Nine children had 29 episodes of SJS or incomplete SJS; all children were male and 8 (88%) were white. Episodes affected mucus membranes with minimal skin involvement. Mycoplasma infections and HLA-B27/-B51 were common.

Symptomatic Congenital Hemangioma and Congenital Hemangiomatosis Associated With a Somatic Activating Mutation in GNA11.

IMPORTANCE: Congenital hemangiomas are uncommon benign vascular tumors that present fully formed at birth. They are rarely associated with transient hematologic abnormalities, which are typically less severe than the Kasabach-Merritt phenomenon associated with kaposiform hemangioendotheliomas. Congenital hemangiomas are typically solitary and have not been reported to occur in a multifocal, generalized pattern. OBJECTIVE: To describe a male infant born with an unusual, large vascular mass complicated by anemia, thrombocytopenia, and disseminated intravascular coagulopathy, as well as innumerable small vascular papules in a generalized cutaneous distribution. DESIGN, SETTING, AND PARTICIPANT: This case report is a descriptive observation of the results of clinical, pathologic, and genetic studies performed in a single male infant observed for 2 years (May 2013 to June 2015) for vascular anomalies at a tertiary care referral center. MAIN OUTCOMES AND MEASURES: Histopathologic, immunohistochemical, and genetic study results of tumor specimens and saliva. RESULTS: Careful pathologic study of 3 tumor specimens revealed similar lobular proliferations of bland endothelial cells. Lesional vessels did not express GLUT1 or the lymphatic marker D2-40, whereas WT1 was expressed. A somatic c.A626C, p.Q209P mutation in the GNA11 gene was identified in tumoral tissue. CONCLUSIONS AND RELEVANCE: These findings support a unifying diagnosis of congenital hemangioma for these vascular tumors. To date, this is the first-reported case of a hemangiomatosis presentation of congenital hemangioma. In addition to highlighting this novel phenotype, this case indicates the rare association of congenital hemangioma with hematologic abnormalities and verifies somatic activating mutations as the underlying cause of congenital hemangioma.

Hereditary Progressive Mucinous Histiocytosis: New Insights Into a Rare Disease.

Hereditary progressive mucinous histiocytosis is a rare, benign, skin-limited form of non-Langerhans cell histiocytosis. We report on a 5-year-old boy who presented in infancy with self-resolving dermal nodules but later developed persistent and progressive erythematous papules on the face and scalp. Histologic evaluation revealed dermal aggregates of S-100/CD1a-negative histiocytes with abundant mucin. We present this case to highlight the evolution of the lesional morphology in infancy and early childhood and to stress the importance of histology in confirming this rare disorder.

Spiny Papules in an Immunosuppressed Child.

Trichodysplasia spinulosa is a rare folliculocentric polyomavirus infection observed in the setting of immunosuppression. We report a 7-year-old boy with pre-B-cell lymphoblastic leukemia who presented with folliculocentric spiny papules on the face. Histologic evaluation revealed hypertrophic bulbs, an expanded inner root sheath, and numerous brightly eosinophilic trichohyalin granules. We present this case to raise awareness of this rare but recognizable entity and to highlight the availability of appropriate diagnostic and therapeutic modalities.

A visible response to an invisible tattoo.

Invisible (or blacklight) tattoos are fast becoming the trend in the world of tattoo art, and with their rise comes the onset of associated complications. Though there have been many reports of cutaneous reactions to traditional tattoo pigments, literature regarding reactions to invisible tattoos is scarce. We report the case of a 28-year-old man who presented with an inflammatory eruption of 2 months' duration confined to the area of a recently placed invisible tattoo; the eruption was diagnosed as granulomatous dermatitis to a foreign material. Under fluorescent light, a refractile foreign material was identified in the biopsy specimen, which we believe to be melamine, one of the invisible tattoo's five ingredients. Previous cases of cutaneous reactions to invisible tattoos were attributed to polymethylmethacrylate, not a component of the tattoo in this case. To our knowledge, this is the first case implicating melamine as the cause of a granulomatous tattoo reaction. Given the rising popularity of invisible tattoos, we present this case to raise awareness of the risks associated with this alternative tattoo trend.

Case report--papular xanthoma in a 10-year-old female with abnormal lipid profile.

Papular xanthoma is a rare normolipemic xanthomatous skin disease which primarily occurs in adults. While this rare disorder has been documented in children, all but one reported in the literature had a normal lipid profile. We report a 10-year-old girl with multiple papule xanthomas on her face that had an abnormal lipid profile. To our knowledge this is only the second reported case of papular xanthoma in the pediatric population with an abnormal lipid profile.

Nevi in children: a practical approach to evaluation.

Education is the cornerstone of melanoma prevention. Well-child examinations are an excellent opportunity to educate patients and parents about melanoma risk factors and prevention. The only modifiable risk factor for the development of melanoma and acquisition of nevi is level of sun exposure. Every patient who is evaluated for a melanocytic lesion should be educated about sun avoidance during midday, protective clothing including long-sleeved shirts, long pants, and hats with brims, and playing in shaded areas when outdoors. Proper application and appropriate uses of sunscreen should also be reviewed.