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Year : 2018  |  Volume : 19  |  Issue : 2  |  Page : 180-182

Anetoderma-like presentation of cutaneous mastocytosis

1 Department of Dermatology, Sir Ganga Ram Hospital, New Delhi, India
2 Consultant Dermatologist, Skin City Clinic, Pune, Maharashtra, India

Date of Web Publication26-Mar-2018

Correspondence Address:
Priyanka Borde Bisht
Skin City Clinic, Pune Camp, Pune, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijpd.IJPD_41_17

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How to cite this article:
Garg S, Bisht PB. Anetoderma-like presentation of cutaneous mastocytosis. Indian J Paediatr Dermatol 2018;19:180-2

How to cite this URL:
Garg S, Bisht PB. Anetoderma-like presentation of cutaneous mastocytosis. Indian J Paediatr Dermatol [serial online] 2018 [cited 2020 Dec 3];19:180-2. Available from: https://www.ijpd.in/text.asp?2018/19/2/180/211816


A 1½-year-old male child was brought to us with intensely itchy dark or skin-colored lesions since 8 months of age. Mother gives a history that lesions swell up and become reddish on and off and resolve on their own over a few hours or 1–2 days or sometimes after giving syrup Allegra SOS by the pediatrician. Some of them have developed loose skin and dark or light color after healing.

Child did not have any systemic complaints such as abdominal pain/nausea/vomiting/diarrhea/episodes of flushing/headache/syncope.

General and systemic examination was normal. Dermatological examination revealed multiple round to oval normo- to hyper-pigmented macules [Figure 1] and soft pouch-like papules (anetoderma like) [Figure 2] mainly over trunk and few over extremities over flexor as well as extensor aspect, sparing the face, ranging in the size between 0.3 and 1 cm in diameter. On stroking the lesions developed wheel and erythema (Darier's sign positive) [Figure 3].
Figure 1: Normo- to hyper-pigmented macules over trunk

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Figure 2: Soft pouch-like papules (anetoderma like) over back

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Figure 3: Wheel and erythema on stroking the lesions (Darier's sign positive)

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Complete blood count showed Hb: 8.4 g/dl and raised total leukocyte count (18,600 cells/cumm). Ultrasonography (abdomen) did not reveal hepatomegaly or splenomegaly. Serum tryptase levels and urinary histamine levels were within normal range.

Histopathology of the lesion taken from trunk showed normal epidermis with band of inflammatory infiltrate in the upper dermis, consisting predominantly of mastocytes showing cytoplasmic granules on toluidine blue stain, with fragmentation of elastic fibers in the papillary dermis [Figure 4].
Figure 4: Inflammatory infiltrate in the upper dermis of mastocytes with cytoplasmic granules on and fragmentation of elastic fibers in the papillary dermis

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Parents were counseled regarding self-limiting nature of the disease and to avoid trigger factors such as pressure/friction/physical/emotional exertion/extreme temperature changes and SOS use of oral antihistamines under the supervision of pediatrician/dermatologist.

Urticaria pigmentosa is the most common type of mastocytosis seen in children, in 70%–90% of cases. Mastocytoma is seen in 10%–30% cases. Diffuse cutaneous mastocytosis is a rare variant and accounts to 1%–3% cases.[1]

Atypical variants of mastocytosis include bullous, pseudoxanthomatous, giant inguinal and suprapubic masses, and blaschkoid pattern of mastocytosis have been described in the literature.[2],[3],[4]

Episodic, unilateral, swelling of the labia majora that was discovered to be an unusual presentation of mastocytosis has been reported in two female children.[5]

Anetodermic mastocytosis has been reported in international literature and authors discuss that elastic and collagen fibers in the mastocytosis lesions degenerate and result in a laxity of skin causing anetoderma.[6],[7]

However, anetodermic presentation of mastocytosis has not been reported in Indian literature yet and this case highlights the fact that mastocytosis is frequently misdiagnosed because of its rarity and variable clinical presentation, which often mimics other conditions.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

The study was supported by the Department of Dermatology, Sir Ganga Ram Hospital, Rajinder Nagar, New Delhi.

Conflicts of interest

There are no conflicts of interest.

  References Top

Srinivas SM, Dhar S, Parikh D. Mastocytosis in children. Indian J Paediatr Dermatol 2016;16:57-63.  Back to cited text no. 1
Rajesh J, Dogra S, Verma S, Mohanty SK, Handa S. Diffuse cutaneous mastocytosis: Pseudoxanthomatous variant. J Dermatol 2002;29:354-6.  Back to cited text no. 2
Murphy M, Walsh D, Drumm B, Watson R. Bullous mastocytosis: A fatal outcome. Pediatr Dermatol 1999;16:452-5.  Back to cited text no. 3
Mehta S, Masatkar V, Khare AK, Mittal A, Gupta LK. Blaschkoid mastocytosis. Indian J Dermatol Venereol Leprol 2015;81:72-3.  Back to cited text no. 4
[PUBMED]  [Full text]  
Roy J, Metry DW, Hicks J, Morgan AJ, Heptulla RA. An unusual presentation of mastocytosis: Unilateral swelling of the vulva. Pediatr Dermatol 2005;22:554-7.  Back to cited text no. 5
Kalogeromitros D, Gregoriou S, Makris M, Georgala S, Kempuraj D, Theoharides TC. Secondary anetoderma associated with mastocytosis. Int Arch Allergy Immunol 2007;142:86-8.  Back to cited text no. 6
Del Pozo J, Pimentel MT, Paradela S, Almagro M, Martínez W, Fonseca E. Anetodermic mastocytosis: Response to PUVA therapy. J Dermatolog Treat 2007;18:184-7.  Back to cited text no. 7


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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