Xanthoma disseminatum: improvement in disfiguring facial lesions with cladribine

Gupta, Vishal; Khaitan, Binod K.; Patra, Suman; Yadav, Rajni

doi:10.1111/jdv.13289

Cited by 10 publications

(16 citation statements)

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“…Xanthoma disseminatum (XD) is a rare, non‐Langerhans cell histiocytosis characterized by multiple red to brown papules and nodules involving the skin and mucosal membranes . XD is very rare, with approximately 100 cases reported in the literature . The onset of XD ranges from as early as 8 months to as late as 85 years, and is commonest in young adult men, with a male : female ratio of 2.4 : 1 .…”

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“…These often initially appear in flexural sites such as the axillae. The lesions tend to have a symmetrical distribution over the body . Mucous membrane involvement occurs in 40–60% of patients.…”

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“…Mucous membrane involvement occurs in 40–60% of patients. XD has persistent, progressive and selflimiting forms, which are classified on the basis of disease progression and prognosis . Spontaneous resolution has also been reported in one‐third of patients over a period of 5–30 years …”

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“…XD is thought to be a primary reactive proliferation of macrophages with secondary lipid accumulation . Associations with multiple myeloma, Waldenstrom macroglobulinaemia, monoclonal gammopathy, hypothyroidism or hyperthyroidism, transient mild diabetes insipidus, epilepsy, hydrocephalus and cerebellar ataxia have been reported . Treatment with glucocorticoids, fibrates, cyclophosphamide or azathioprine fail to produce long‐lasting beneficial effects in most cases.…”

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“…Treatment with glucocorticoids, fibrates, cyclophosphamide or azathioprine fail to produce long‐lasting beneficial effects in most cases. Surgical excision or laser therapy can improve the physical and functional appearance, but they do not prevent relapses …”

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Cladribine is a promising therapy for xanthoma disseminatum

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Xanthoma disseminatum (XD) is a rare, nonLangerhans cell histiocytosis characterized by multiple red to brown papules and nodules involving the skin and mucosal membranes.1,2 XD is very rare, with approximately 100 cases reported in the literature. 2-5The onset of XD ranges from as early as 8 months to as late as 85 years, 3 and is commonest in young adult men, with a male : female ratio of 2.4 : 1.4 XD appears to occur sporadically, with no known hereditary factors identified.2,4 XD is a disease with a chronic and progressive course. It presents a therapeutic challenge. We present a patient with the typical clinical and histological features of XD who was successfully treated with cladribine.A 19-year-old woman presented an 8-year history of an asymptomatic eruption, which had first appeared slowly on the anterior surface of the chest, and then progressed to involve the face, neck, arms and other flexural surfaces. Her medical history was otherwise unremarkable.Physical examination revealed yellow to red-brown coloured papules and nodules on the patient's face, neck and shoulder, the anterior and posterior surface of her chest, and the mucosal surfaces, along with verrucous tumours on the flexural surfaces of the inguinal area, popliteal fossae and axillary folds (Fig. 1a).Laboratory investigations, including full blood count, erythrocyte sedimentation rate, levels of C-reactive protein, urea and electrolytes, lipid profile, protein electrophoresis, urinalysis, 24-hour urine protein test, and chest radiography were all normal.Biopsies taken from the oral mucosa, anterior surface of the chest and inguinal region showed histopathological changes characteristic of XD (Fig. 2), thus the diagnosis of XD was made.We treated the patient with cladribine (2-chlorodeoxyadenosine) 0.14 mg/kg/day for 5 days each week, repeated every month. 4 After 7 months of treatment, many of the papular lesions had disappeared, leaving slightly hyperpigmented macules, but the rapid flattening process that took place in flexural areas resulted in an appearance of the loose skin (Fig. 1b). Cladribine therapy was well-tolerated, with few adverse effects. No new lesions developed during 2 years of follow-up.XD is characterized by multiple, asymptomatic, yellowish or red to brown papules, nodules and tumours. These often initially appear in flexural sites such as the axillae. The lesions tend to have a symmetrical

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Intertriginous Multiple Reddish-Brown Papulonodules

Wang

2018

JAMA Dermatol

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A teenaged male presented with a 7-year medical history of numerous asymptomatic reddish-brownish papules, nodules, and plaques over his whole body. The papules began from his bilateral eyelid margins and extended to his trunk and extremities. He also reported a 7-year medical history of polydipsia and polyuria. Physical examination revealed reddishbrown papules, nodules, and plaques on his periocular areas, perioral areas, neck (Figure, A), shoulder, axillary folds, cubital fossa, wrists, palms, umbilical region, lateral abdomen, inguinal area, anogenital area (Figure, B), popliteal fossa, and ankles. The patient had also been diagnosed with central diabetes insipidus, growth retardation, and hypothyroidism. A skin biopsy was performed at the time of the initial evaluation (Figure, C and D).Clinical image A Histopathologic image C Clinical image B Histopathologic image D Figure. A, Reddish-brown papules and plaques on the neck. B, Reddish-brown papules and plaques on the umbilical region, lateral abdomen, inguinal area, and genital area. C, Hematoxylin-eosin stain (original magnification ×100). D, Hematoxylin-eosin stain (original magnification ×200). WHAT IS YOUR DIAGNOSIS? A. Eruptive xanthomas B. Langerhans' cell histiocytosis C. Juvenile xanthogranuloma D. Xanthoma disseminatum Clinical Review & Education

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