Late manifestation of diabetes insipidus in “pure” cutaneous Langerhans cell histiocytosis

Hoeger, Peter H.; Janka‐Schaub, Gritta; Mensing, H.

doi:10.1007/s004310050653

Cited by 12 publications

(7 citation statements)

References 21 publications

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“…We found hyperpigmentation in one patient. Scars (confined to areas of former severe skin involvement) were noted in in six of 20 patients (30%), but such changes can be observed in LCH patients with severe skin disease irrespective of therapy,3 4 32 and are therefore considered a consequence of the disease, not the treatment.…”

Section: Discussionmentioning

confidence: 96%

Long term follow up of topical mustine treatment for cutaneous Langerhans cell histiocytosis

Hoeger

Nanduri²,

Harper³

et al. 2000

Archives of Disease in Childhood

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Background and objectives-Skin lesions in Langerhans cell histiocytosis (LCH) are often painful and diYcult to treat. Topical application of nitrogen mustard (0.02% mechlorethamine hydrochloride, mustine), an alkylating cytostatic agent, has been shown to be eVective. There is, however, concern about potentially harmful long term side eVects. Study design-In a retrospective study 20 children with LCH (average extent of initial skin involvement: 16.4% body surface) were followed up for an average of 8.3 years after completion of topical mustine therapy. They had received a total of 34 courses (mean duration 14.2 weeks) of topical mustine. Disease status on follow up was assessed according to the Histiocyte Society classification. Results-After mustine was introduced, 16 patients were able to discontinue systemic steroids and/or chemotherapy. Topical mustine was well tolerated in 18 patients, but two developed irritant dermatitis. On follow up, the disease was inactive in 10 patients. Among the children with active disease, six had mild skin disease and four had progressive disease, two of them with skin lesions unresponsive to mustine treatment. Scars confined to areas of formerly active skin disease were found in six patients. There was no evidence of premalignant or malignant skin disease in the treated areas. Conclusion-Topical mustine is an eVective and safe treatment for skin disease in most children with LCH. Residual scarring was probably a result of the disease itself rather than to mustine. Although no evidence of skin cancer was found in this study, continued long term follow up is advisable. (Arch Dis Child 2000;82:483-487)

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Section: Discussionmentioning

confidence: 96%

Long term follow up of topical mustine treatment for cutaneous Langerhans cell histiocytosis

Hoeger

Nanduri²,

Harper³

et al. 2000

Archives of Disease in Childhood

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“…CSHLCH usually has a favorable prognosis, but some cases were demonstrated to have a relapse of skin or visceral involvement after apparent resolution of clinical disease. 8,9 According to the review by Ricart et al, our case did not fulfill the good prognosis criteria in LCH. 2 One of the reasons is that Birbeck granules and laminated dense bodies were not present within the same cells.…”

Section: An Incidence Of Neonatal Langerhans Cell Histiocytosis (Lch)mentioning

confidence: 70%

“…Even for a case without symptoms, we suggest that systemic investigation, including chest CT, is recommended to evaluate this disorder. CSHLCH usually has a favorable prognosis, but some cases were demonstrated to have a relapse of skin or visceral involvement after apparent resolution of clinical disease 8,9 . According to the review by Ricart et al ., our case did not fulfill the good prognosis criteria in LCH 2 .…”

Section: Discussionmentioning

confidence: 99%

Congenital self‐healing LCH: A case with lung lesions and review of the literature

Nakashima¹,

Onoe²,

Tashiro

et al. 2010

Pediatrics International

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Congenital self-healing Langerhans cell histiocytosis (CSHLCH), a rare benign disorder, is initially seen at birth or in the newborn period. In 1973, Hashimoto and Pritzker first reported this disease 1 and since then more than 30 cases have been reported in Japan. CSHLCH is characterized by eruptions of multiple papules and nodules that resolve spontaneously without involvement of other organs. 1,2 Here we present a case of a neonate who was born with multiple skin erosions mimicking varicella and lung nodules that regressed spontaneously. Case reportA female infant, who weighed 2980 g, was born by spontaneous vaginal delivery at 38 weeks' gestation to a healthy mother after an uncomplicated pregnancy. The mother had no contributory family history and was not a smoker. The infant was transferred to our hospital on the first day of life because of multiple skin erosions and vesicles that presented at birth. On examination at our hospital, her temperature was 36.4°C, her pulse rate was 140 b.p.m., her respiratory rate was 40 per minute, and her blood pressure was 69/38 mmHg. She had multiple cutaneous erosions and varicelliform vesicles of 5 to 10 mm in diameter, which were scattered over her whole body including her scalp (Fig. 1). The mucomembranous surface, lymph nodes, and liver were not involved. Complete blood cell count and blood chemistry results were as follows: white blood cells, 17.8 ¥ 10 9 /L with 73% segmented neutrophils, 21% lymphocytes, and 6% monocytes; red blood cells, 4.89 ¥ 10 12 /L; hemoglobin, 17.9 g/dL; hematocrit, 54.6%; platelets, 353 ¥ 10 9 /L; lactic dehydrogenase, 541 IU/L; aspartate aminotransferase, 48 IU/L; and alanine aminotransferase, 16 IU/L. Chest X-ray showed no lesions in either lung. A lumbar puncture was performed, and an examination of the cerebrospinal fluid was normal. The patient was suspected to have congenital herpes simplex virus (HSV) or varicella-zoster virus (VZV) infections.However, direct immunofluorescence assays from basal membrane of vesicles for HSV and VZV were negative. Serological studies were also negative on both HSV and VZV. She was treated with acyclovir for a week until HSV and VZV were ruled out. On the 19th day after birth, histopathological cutaneous examination of her back lesion was performed. It revealed numerous mononuclear cells with kidney-shaped nuclei. Immunochemistry analysis showed that cells were positive for CD1a and S-100 protein: both were characteristic of Langerhans cells. Electron microscopic examination revealed Birbeck granules in the infiltrating cells (Fig. 2), but laminated dense bodies were not found in the same cells. The skin lesions soon regressed with incrustation, and completely disappeared spontaneously within a month (Fig. 1). Therefore, we diagnosed this case as congenital self-healing Fig. 1 Multiple cutaneous erosions and varicelliform vesicles were scattered over the whole body (day 0). By day 16 they had almost disappeared spontaneously.

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“…The systemic examination was negative, and the infant was treated with two courses of vinblastine and methylprednisolone. The eruption cleared at age 18 months, but at 24 months the infant developed diabetes insipidus (8). Eosinophilic granuloma of the skull developed in an adolescent who had widespread papulovesicular CSHR as an infant (Weinberg S, personal communication).…”

Section: Discussionmentioning

confidence: 99%

Congenital Self‐Healing Reticulohistiocytosis with Eye Involvement

Zaenglein¹,

Steele

Kamino

et al. 2001

Pediatric Dermatology

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Congenital self-healing reticulohistiocytosis (CSHR) represents the benign end of the spectrum of Langerhans cell histiocytoses, with spontaneous resolution of lesions within the first year of life. However, involvement of organ systems other than the skin has been described occasionally and recurrence of disease at sites distant from the skin has been documented. We report a case of CSHR with eye involvement that spontaneously resolved concurrent with resolution of skin lesions. Because multiple organ systems can be involved and recurrences are possible, long-term follow-up of these patients is indicated.

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Late manifestation of diabetes insipidus in “pure” cutaneous Langerhans cell histiocytosis

Abstract: "Pure cutaneous" LCH, frequently also referred to as congenital self-healing LCH, is a variable disorder which may be complicated by late organ involvement. Close follow up and thorough diagnostic evaluation is therefore mandatory.

Cited by 12 publications

References 21 publications

Long term follow up of topical mustine treatment for cutaneous Langerhans cell histiocytosis

Long term follow up of topical mustine treatment for cutaneous Langerhans cell histiocytosis

Congenital self‐healing LCH: A case with lung lesions and review of the literature

Congenital Self‐Healing Reticulohistiocytosis with Eye Involvement

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