Reddish ulceration on a neonate's face - Clinical Advisor

Reddish ulceration on a neonate’s face

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  • August 2015 Dermatology Clinic

    August 2015 Dermatology Clinic

A 3-day-old male who is otherwise healthy presents with a single lesion on the right side of his face. The child’s mother reported that the neonate had a “red dot” on her face at birth. When the infant was aged 1 day, the red dot had progressed to ulceration. The child’s medical history was not significant. He had been delivered vaginally, at full term, and following an uncomplicated pregnancy. The physical examination was normal. There were no signs of hepatosplenomegaly or lymphadenopathy.



This The Clinical Advisor CME activity consists of 3 articles. To obtain credit, read Large patch of coalescing gray-brown macules and Small, vascular, red and violet lesions. Then take the post-test here.


This The Clinical Advisor CME activity consists of 3 articles. To obtain credit, read Large patch of coalescing gray-brown macules and Small, vascular, red and violet lesions. Then take the post-test here.Congenital self-healing reticulohistiocytosis (CSHRH), also known as Hashimoto-Pritzker disease,...

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This The Clinical Advisor CME activity consists of 3 articles. To obtain credit, read Large patch of coalescing gray-brown macules and Small, vascular, red and violet lesions. Then take the post-test here.


Congenital self-healing reticulohistiocytosis (CSHRH), also known as Hashimoto-Pritzker disease, was first reported in 1973 when an infant was born with 30 to 35 red-brown nodules that upon biopsy proved to be characterized by an intradermal proliferation of reticulohistiocytes. These nodules subsequently disappeared within three and a half months and did not recur.1 There are now more than 125 published cases of CSHRH. 


CSHRH occurs in otherwise well-appearing neonates and equally affects both males and females. The associated lesions are typically present at birth. However, they may also appear within a few months after birth. The lesions most commonly present as multiple 0.5-mm to 2-cm papules or nodules that evolve into crusted papules or vesicles.2,3 Single lesions, as was seen in the patient in the introductory case, occur in approximately 20% of cases.2 The initial eruptions are painless and vary in color from pink to red to brown. Lesions are found most commonly on the head, trunk, or extremities; in rare cases, they are found on the palms or groin.2 Typically, the lesions spontaneously regress within three months. 


CSHRH is a congenital form of a group of diseases referred to as Langerhans cell histiocytosis (LCH). These diseases result from an abnormal monoclonal proliferation of the antigen-presenting Langerhans cells. This group of diseases includes Hand-Schüller-Christian disease, Letterer-Siwe disease, eosinophilic granuloma, and CSHRH.4 Cutaneous manifestations are the most common initial presenting sign of LCH in children younger than age 2 years.5 LCH may involve multiple internal organs, which most commonly include the bone and skin but may include the liver, thymus, or lungs. It has been suggested that CSHRH and multisystem LCH are a spectrum of diseases.5 Based solely on initial presentation, systemic forms of LCH cannot always be differentiated from CSHRH. Furthermore, due to the spontaneous regression of CSHRH lesions, it is thought that the actual prevalence of the disease is much greater than what has been reported. 


Diagnosis of CSHRH is made with a biopsy of the lesion showing Langerhans histiocytes in the dermis. These cells stain positively for CD1a and S100 proteins, and electron microscopy reveals their characteristic Birbeck granules.6 The workup for patients with suspected CSHRH includes physical examination for lymphadenopathy or hepatosplenomegaly, complete blood count (CBC) with differential, liver function tests, coagulation studies, chest x-ray for lung involvement, urine osmolality, and skeletal survey. This complete workup, accompanied by routine follow-up visits over the next two years, allows for the exclusion of other forms of LCH that have systemic involvement. 


When CSHRH presents as a solitary lesion, the differential diagnosis includes hemangioma, infantile myofibroma, infantile fibrous hamartoma, juvenile xanthogranuloma, and congenital Spitz nevus.3 When CSHRH presents as multiple erythematous papules, the differential diagnosis includes congenital syphilis, blue rubber bleb nevus syndrome, blueberry muffin baby, congenital leukemia cutis, and infantile hemangiomas. Histopathology following skin biopsy confirms the diagnosis of CSHRH. 


The most common outcome after CSHRH is changes in skin pigmentation.2 However, it is thought that CSHRH can develop into disseminated LCH.5 Therefore, these patients must be closely monitored for disease recurrence and systemic involvement.6 The rate of recurrent outbreaks of lesions is approximately 10%.2 When the disease recurs, there is a 30% mortality rate due to systemic involvement.2 Overall, there is a 3% mortality rate in neonates with CSHRH.2

CSHRH that is limited to the skin does not require treatment. The patient should be followed for two years with laboratory studies repeated at 6 months and imaging studies repeated based on clinical suspicion for systemic involvement or disease relapse. 


In our case, a biopsy of the patient’s lesion was obtained. Histopathology revealed dermal infiltration with Langerhans histiocytes. A diagnosis of LCH was made. A CBC with differential, liver function tests, urine analysis, coagulation studies, chest x-ray, and skeletal survey were obtained. All of his laboratory and imaging studies returned normal. At a follow-up appointment three months later, his skin lesion had partially regressed. A diagnosis of CSHRH was made based on the regression of his initial lesion and absence of extracutaneous manifestations. He received no initial therapy. At his six-month follow-up appointment, he had repeat laboratory tests and additional imaging studies, all of which were within normal limits. There were no dermatologic signs of disease recurrence or laboratory abnormalities at six-month follow up. The child will be followed in the pediatric dermatology clinic for two years.


Tests to include for patients with suspected CSHRH

Physical examination for lymphadenopathy or hepatosplenomegaly
Complete blood count with differential
Liver function tests
Coagulation studies
Chest x-ray for lung involvement
Urine osmolality
Skeletal survey

Danielle Brown, BA, is a medical student and Maura Holcomb, MD, is a dermatology resident at Baylor College of Medicine in Houston.



This The Clinical Advisor CME activity consists of 3 articles. To obtain credit, read Large patch of coalescing gray-brown macules and Small, vascular, red and violet lesions. Then take the post-test here.


References


  1. Hashimoto K, Pritzker MS. Electron microscopic study of reticulohistiocytoma. An unusual case of congenital, self-healing reticulohistiocytosis. Arch Dermatol. 1973;107(2):263-270. 

  2. Larsen L, Merin MR, Konia T, Armstrong AW. Congenital self-healing reticulohistiocytosis: Concern for a poor prognosis. Dermatol Online J. 2012;18(10):2. Available at escholarship.org/uc/item/78v7z5w4 

  3. Kim JE, Kim BJ, Kang H. Solitary congenital erosion in a newborn: Report of a solitary congenital self-healing reticulohistiocytosis. Ann Dermatol. 2014;26(2):250-253. Available at ncbi.nlm.nih.gov/pmc/articles/PMC4037681 

  4. Rubio-González B, García-Bracamonte B, Ortiz-Romero PL, et al. Multisystemic Langerhans cell histiocytosis mimicking diffuse neonatal hemangiomatosis. Pediatr Dermatol. 2014;31(3):e87-e89. 

  5. Kapur P, Erickson C, Rakheja D, et al. Congenital self-healing reticulohistiocytosis (Hashimoto-Pritzker disease): Ten-year experience at Dallas Children’s Medical center. J Am Acad Dermatol. 2007;56(2):290-294. 

  6. Mandel VD, Ferrari C, Cesinaro AM, et al. Congenital “self healing” Langerhans cell histiocytosis (Hashimoto-Pritzker disease): A report of two cases with the same cutaneous manifestations but different clinical course. J Dermatol. 2014;41(12):1098-1101. 


All electronic documents accessed on August 4, 2015.



This The Clinical Advisor CME activity consists of 3 articles. To obtain credit, read Large patch of coalescing gray-brown macules and Small, vascular, red and violet lesions. Then take the post-test here.


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