๐Ÿ—‚ ็ธฝ็›ฎ้Œ„ ๏ฝœ ๐Ÿ“– ่‹ฑๆ–‡ๅŽŸๆ–‡๏ผˆๆœฌ็ฏ‡๏ผ‰ ๏ฝœ ๐Ÿ“ ๅฎŒๆ•ด็ฟป่ญฏ ๏ฝœ โญ ็ฒพ่ฏ็ญ†่จ˜

Diffuse cutaneous mastocytosis

Diffuse cutaneous mastocytosis

This is very rare and affects mainly children and only rarely adults.13,46โ€“48 The skin is erythrodermic, often itchy, and, due to the widespread infiltrate of mast cells, has a thick doughy or boggy consistency, with accentuation of surface markings. Occasionally, the skin may have a red, yellow-brown, โ€˜peau dโ€™orangeโ€™, or grain leather (โ€˜peau chagrineโ€™) appearance.38 Lichenification is sometimes evident. Urtication and blister formation following mild trauma are usual.38,46 Generalized blistering may occur, sometimes mimicking staphylococcal scalded skin syndrome or bullous erythema multiforme.49โ€“51 Diffuse cutaneous mastocytosis commonly resolves by the

third to fifth years.11 Systemic symptoms include flushing, hypotension, shock, and diarrhea.11

Fig. 29.350 Urticaria pigmentosa: note the generalized distribution of the lesions. From the collection of the late N.P. Smith, MD, the Institute of Dermatology, London, UK.