Congenital Self-healing Langerhans Cell Histiocytosis Research Articles

Congenital self-healing Langerhans cell histiocytosis presented as ‘purpura-like skin lesions'

At birth, a newborn boy had disseminated purplish patches varying in size from 3 to 5 mm (Fig. 1A). There was no involvement of the mucomembranous surface, lymph nodes, and liver. He was active and stable. A prompt punch biopsy of a representative lesion revealed intensive infiltration of the Langerhans cells that were positive for CD1α and S-100 (Fig. 1B–D). The patient was diagnosed with congenital Langerhans cell histiocytosis (LCH). Laboratory results including complete blood cell count; blood chemistry results; coagulation; antibodies for HAV, HBV, HCV, rubella, HSV, HIV, and syphilis; and blood cultures were normal.

Congenital Langerhans Cell Histiocytosis Presenting as Neonatal Papulovesicular Eruption

Congenital self-healingLangerhans cell histiocytosis (CSHLCH) is a rare type of Langerhans Cell Histiocytosis, presenting at birth or during the neonatal period. It is usually characterized by the eruption of multiple, discrete, red-brown papules or nodules which may increase in size and number during the first few weeks of life with spontaneous regression.Congenital LCH has rarely been reported to present as a papulovesicular eruption at birth. Here we describe a male baby presenting with papulovesicular eruption at birth who rapidly developed pulmonary infiltrates and multiple osteolytic lesions in skull and long bones after spontaneous regression of cutaneous lesion.

A case of congenital self-healing Langerhans cell histiocytosis

A case of congenital self-healing Langerhans cell histiocytosis

Congenital self-healing reticulohistiocytosis in a newborn: A rare variant

Congenital self-healing reticulohistiocytosis or congenital self-healing Langerhans cell histiocytosis (LCH) or Hashimoto-Pritzker syndrome is a rare, benign, auto involuting form of LCH. It usually presents at birth or soon thereafter with spontaneously regressing multiple or solitary dusky papules or nodules. The actual incidence remains unknown probably because of its benign and self-limiting nature. Herein, we present a similar case present since birth because of its rarity in nature.

Solitary congenital self-healing Langerhans cell histiocytosis: A benign variant of Langerhans cell histiocytosis?

Langerhans cell histiocytosis (LCH) represents a diverse group of diseases with various different clinical presentations and outcomes. We present two cases of solitary CSH-LCH (sCSH-LCH) and highlight certain unique characteristics including the favorable prognosis and lack of documented progression to systemic involvement in reported cases.

Congenital self-healing histiocytosis presenting as blueberry muffin baby: A case report and literature review

Congenital self-healing Langerhans cell histiocytosis (CSHLCH), also called as Hashimoto-Pritzker disease, is a rare, benign variant of histiocytosis. Despite the initial dramatic clinical presentation, affected infants are otherwise healthy and skin lesions disappear spontaneously within several weeks to months. We present a case of CSHLCH presenting as blueberry muffin baby. The lesions appeared in the first week of life and lasted 6 months. The follow-up period was 24 months, without any signs of relapse. At the pediatric dermatology unit of our clinic, during the last 20 years, we had 10 children with Langerhans cell histiocytosis and among them only one with CSHLCH. In the literature, we found only 5 newborns with Langerhans cell histiocytosis presenting as blueberry muffin baby, among them only 4 with self-healing CSHLCH. The early recognition of CSHLCH may spare children from redundant and potentially toxic systemic treatment.

Kongenitale selbst heilende Langerhans-Zell-Histiozytose

A male infant, born at term after an uncomplicated pregnancy, presented with multiple red-brown papules and nodules spread over his body. The infant's general condition was not affected and there was no systemic involvement. After only 6 weeks the lesions had healed completely without recurrence. Based on histological and immunohistochemical findings, the clinical appearance and the self-healing course, the diagnosis of congenital self-healing Langerhans cell histiocytosis was established.

Exploring the Differential Diagnosis of Hemorrhagic Vesicopustules in a Newborn

Hemorrhagic vesicles in a newborn present a challenging differential diagnosis including both infectious and neoplastic disorders. Patients should be evaluated in an efficient manner to arrive at the correct diagnosis as quickly as possible. We present here an interesting case that outlines the methodical workup that ultimately revealed the diagnosis of congenital Langerhans cell histiocytosis. After a diagnosis of Langerhans cell histiocytosis is made, it is important to evaluate the patient thoroughly for systemic involvement. Historically, the diagnosis of congenital self-healing Langerhans cell histiocytosis was used to delineate a benign self-limited disorder limited to the skin with spontaneous resolution during the first several months of life; this disorder may also be referred to as "self-regressive Langerhans cell histiocytosis." However, some newborns with initial skin-only Langerhans cell histiocytosis progress to have multisystem disease after spontaneous resolution has occurred. For this reason, the nomenclature is changing. We suggest using the term "skin-only Langerhans cell histiocytosis." Periodic long-term follow-up is recommended to monitor for relapse or progression to systemic disease.

Congenital Langerhans cell histiocytosis with skin and lung involvement: Spontaneous regression

Congenital self-healing Langerhans cell histiocytosis (CSHLCH) is a rare type of Langerhans Cell Histiocytosis, presenting at birth or during the neonatal period with eruption of multiple, discrete red-brown papules or nodules that undergo spontaneous regression. Systemic signs are generally absent. We describe a 4-month-old infant presenting with reddish brown nodular cutaneous lesions since birth with a past history suggestive of pulmonary involvement. Skin biopsy showed a dermal infiltrate of pleomorphic histiocytes; which were CD1a and S-100 positive, consistent with the diagnosis of CSHLCH. Both pulmonary and cutaneous lesions showed spontaneous resolution.

Disseminated crusted papules in a newborn

Congenital self-healing Langerhans cell histiocytosis (Hashimoto-Pritzker disease) is the rarest form of Langerhans cell histiocytosis, usually confined to the skin and/or mucous membranes. Cutaneous eruption is mostly generalized, papular, nodular or vesicular. Despite impressive clinical presentation in a newborn it infrequently spreads to internal organs (which then portends a grave prognosis, indistinguishable from Letterer-Siwe disease). We presented a full-term newborn, female, 3.3 kg who had a multitude of erythematous and crusted papules, nodules and pseudovesicles distributed all over the body, except for the mucous membranes. A biopsy and haematoxylin--eosin stain revealed dermal infiltration of pleomorphic histiocytes with eosinophilic ground-glass cytoplasm and round to bean-shaped nuclei. Over the next six weeks the eruption gradually subsided leaving no residues, or a few atrophic scars. There is no need for specific treatment of congenital self-healing Langerhans cell-histiocytosis in the absence of multiorgan involvement. However, a close and regular follow-up is necessary to evaluate the children for systemic symptoms and signs.

Diagnostic Electron Microscopy and Immunohistochemistry Part I: Neoplastic Diseases

Diagnostic Electron Microscopy and Immunohistochemistry Part I: Neoplastic Diseases

Congenital Self-Healing Langerhans Cell Histiocytosis: Complementary Role of Immunocytochemical and Ultrastructural Evaluation

Congenital Self-Healing Langerhans Cell Histiocytosis: Complementary Role of Immunocytochemical and Ultrastructural Evaluation

Self-Healing Congenital Langerhans Cell Histiocytosis Presenting as Neonatal Papulovesicular Eruption

Background: Congenital self-healing Langerhans cell histiocytosis (CSHLCH) is a rare condition which may present at birth or during the neonatal period. It is usually characterized by the eruption of multiple, disseminated, red-brown papules and nodules which may increase in size and number during the first few weeks of life. Systemic signs are usually absent except for occasional mild hepatomegaly. Objective: We present a 3.5-kg male infant who presented at birth with numerous diffuse, erythematous, crusted erosions. He was presumed to have congenital herpes simplex virus (HSV) and was started on IV acyclovir. Histopathologic examination revealed a mixed inflammatory infiltrate with numerous histiocytes which were S-100 and peanut agglutin positive consistent with CSHLCH. Further workup did not reveal any signs of systemic involvement. Conclusion: CSHLCH has rarely been reported to present as a papulovesicular eruption at birth. In these cases, a viral etiology is commonly entertained in the differential diagnosis. Despite the spontaneous regression of skin lesions in CSHLCH, close followup is required to evaluate for systemic signs and symptoms associated with latent Letterer–Siwe disease.

Congenital self-healing histiocytosis (Hashimoto-Pritzker).

Congenital self-healing Langerhans cell histiocytosis (CSHLCH) is a rare condition, initially seen at birth or in the neonatal period, with generalized papules, vesicles, or nodules. Affected infants are otherwise well and the skin lesions tend to involute spontaneously within weeks to months. Twelve patients with CSHLCH were seen from 1989 to 1998. Eight patients were girls and four were boys and all presented with lesions at birth which disappeared 1-3 months later. The lesions consisted of numerous brownish-red papules, papulovesicles, crusts, and nodules distributed on the face, limbs, palms, and soles. Two patients had oral mucosal lesions, and one had ulcerated lesions that evolved leaving hypochromic macules. Light microscopy showed a histiocytic infiltrate in the papillary dermis with epidermotrophism. Two cases were studied by electron microscopy: the Langerhans cells showed Birbeck granules and laminated corpus in their cytoplasm. Immunomarking with S100 protein was performed in all 12 patients and was positive. CD1 was also tested in four cases and was positive. Because CSHLCH is a rare condition, we emphasize that, although it is usually a benign, self-limited entity, careful evaluation for systemic disease must be performed and long-term follow-up must be carried out to detect evidence of relapse or progression of the disease; this is essential when treating these patients.

Congenital “self-healing” Langerhans cell histiocytosis: The need for long-term follow-up

Congenital self-healing Langerhans cell histiocytosis (CSHLCH) is a rare disorder initially seen at birth or in the newborn period with spontaneously involuting skin lesions. In contrast to other forms of Langerhans cell histiocytosis in infancy, such as Letterer-Siwe disease, the prognosis of CSHLCH is generally good. We describe four children with widespread CSHLCH at birth. In all patients a congenital infection was initially suspected. All patients, except one who had transient lower extremity edema and pulmonary symptoms, had an uncomplicated neonatal course, and in all patients skin lesions resolved spontaneously. Two patients showed no further evidence of disease, but one had a cutaneous relapse at age 3 months, and in another a bony relapse that required systemic therapy developed at 6 months of age. CSHLCH is usually a benign, self-limited condition, but careful evaluation for systemic disease must be performed. Long-term follow-up for evidence of relapse or progression of disease is essential.

Congenital self-healing Langerhans cell histiocytosis with persistent cellular immunological abnormalities

We describe an infant who presented at birth with numerous haemorrhagic and encrusted skin lesions, hepatomegaly, lymphadenopathy, raised hepatic transaminases, leucopenia and thrombocytopenia. The diagnosis of Langerhans cell histiocytosis was confirmed by immunohistochemistry, which demonstrated the presence of CD1, S-100 and DR positive cells in the skin infiltrate. The skin lesions resolved spontaneously after 6 weeks but recurred at 3 months and again were self involuting with resolution by 9 months. Persistent circulating T-cell abnormalities, including T-cell lymphopenia and the presence and persistence of peripheral blood CD1 + cells were noted throughout the first year of life.