Diffuse Cutaneous Mastocytosis: Pseudoxanthomatous Variant

Jain, Rajesh; Dogra, Sunil; Verma, Shikha; Mohanty, Sambit K.; Handa, Sanjeev

doi:10.1111/j.1346-8138.2002.tb00280.x

Cited by 18 publications

(5 citation statements)

References 7 publications

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“…Moreover, we observed that the skin symptoms appeared in age‐dependent manner. Extensive bullous lesions predominated in infancy, whereas diffuse infiltration of the skin developed further in the course of the disease as it has been previously reported 9,17 …”

Section: Discussionsupporting

confidence: 58%

Diffuse cutaneous mastocytosis: analysis of 10 cases and a brief review of the literature

Lange

Niedoszytko

Nedoszytko

et al. 2011

Acad Dermatol Venereol

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Section: Discussionsupporting

confidence: 58%

Diffuse cutaneous mastocytosis: analysis of 10 cases and a brief review of the literature

Lange

Niedoszytko

Nedoszytko

et al. 2011

Acad Dermatol Venereol

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“…Childhood disease may resolve spontaneously. With advancing age, there is risk of extracutaneous involvement and of development of malignancies 5 . Although upon treatment, itching and lesions have shown improvement in this case, the disease may persist or even worsen and therefore long‐term prognosis remains guarded.…”

Section: Discussionmentioning

confidence: 84%

“…This multinodular nonpigmented variety of mastocytosis is extremely rare. In this variety, as against a classic Darier's sign, only erythema without urtication is elicited by rubbing 5 . Skin biopsy reveals dense dermal infiltrate of mast cells.…”

Section: Discussionmentioning

confidence: 94%

See 1 more Smart Citation

Pseudoxanthomatous mastocytosis

Srivastava¹,

Chand²,

Singh³

2007

Int J Dermatology

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A 50‐year‐old woman presented with itchy, multiple, flat, yellowish, waxy papules and plaques diffusely involving her face, trunk, and upper extremities for 5 years (Fig. 1). Itching was moderate to severe in intensity and was episodic, without any identifiable aggravating or relieving factors. On rubbing the lesions with a blunt object erythema was elicited. Her ophthalmological examination was normal. Her complete hemogram was normal along with other routine investigations. Patient's slit skin smear for acid‐fast bacilli and rk39 dip‐stick ELISA for post‐kala‐azar dermal leishmaniasis were negative. Ultrasound examination of her abdomen was normal and skeletal survey showed no evidence of bony changes. Histological examination showed diffuse dense infiltrate of numerous mast cells filling up the papillary dermis and most of upper‐ and mid‐reticular dermis. Overlying epidermis showed moderate epidermal hyperplasia and hyperpigmentation. There was sparse scattering of neutrophils within the infiltrate. Extracellular mast cells granules were also seen (Fig. 2). Diagnosis of diffuse cutaneous mastocytosis of pseudoxanthomatous variety was made. 1 Pretreatment photographs of the patient; (a) face; (b) trunk 2 Histopathology showing dense diffuse infiltrate of numerous mast cells filling up the papillary dermis and most of the upper and mid‐reticular dermis (H&E; a, ×100; b, ×400) The patient was prescribed treatment in the form of H1 and H2 blockers (fexofenadine and ranitidine) and trimethylpsoralen and ultraviolet A (PUVA) photochemotherapy. After 2 months of treatment, there has been marked improvement in the appearance and itching.

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“…In a study on 17 mastocytomas, a pseudoxanthomatous aspect has been reported in up to 40% of cases [6] . In the pseudoxanthomatous variant of diffuse cutaneous mastocytosis there are numerous yellowish pap-ules or nodules associated with the other cutaneous signs [8] . Histologically, in pseudoxanthomatous forms, there is a typical dense mast cell infi ltrate in the deep dermis that might explain the yellow colour of the lesions [9] .…”

Section: Discussionmentioning

confidence: 99%