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Year : 2021  |  Volume : 22  |  Issue : 1  |  Page : 56-58

Localized Pseudoxanthomatous Mastocytosis of Vulva

Department of Dermatology, Mahatma Gandhi Medical College and Hospital, Jaipur, Rajasthan, India

Date of Submission22-Jan-2020
Date of Decision13-Feb-2020
Date of Acceptance09-Jun-2020
Date of Web Publication31-Dec-2020

Correspondence Address:
Shivi Nijhawan
7 Dha 15, Jawahar Nagar, Jaipur, Rajasthan
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijpd.IJPD_14_20

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Cutaneous mastocytosis (CM) is a mast cell proliferative disorder that is common in the pediatric age group. Common entities are urticaria pigmentosa, solitary mastocytoma, telangiectasia macularis eruptive perstans. and diffuse CM. Pseudoxanthomatous mastocytosis presents in the form of yellowish nodular lesion resembling xanthoma and is a rare variant and not reported much in the literature. We, hereby report a rare case of localized vulvar pseudoxanthomatous mastocytosis in a 10-year-old female child.

Keywords: Cutaneous mastocytosis, Localized vulvar pseudoxanthomatous mastocytosis, pediatric mastocytosis

How to cite this article:
Nijhawan S, Nijhawan M, Agarwal S. Localized Pseudoxanthomatous Mastocytosis of Vulva. Indian J Paediatr Dermatol 2021;22:56-8

How to cite this URL:
Nijhawan S, Nijhawan M, Agarwal S. Localized Pseudoxanthomatous Mastocytosis of Vulva. Indian J Paediatr Dermatol [serial online] 2021 [cited 2021 Apr 22];22:56-8. Available from: https://www.ijpd.in/text.asp?2021/22/1/56/305801

  Introduction Top

Mastocytosis is a clonal disorder of mast cells, characterized by their proliferation and accumulation in skin, bone marrow, liver, spleen, and lymph nodes.[1] The first description of mastocytosis was made by Nettleship and Tay in 1869.[2]

The WHO classification of mastocytosis, 2008,[3] has classified these heterogeneous groups of disorders into cutaneous and systemic mastocytosis.

Cutaneous mastocytosis (CM) is the most common presentation and can be subclassified into three clinical variants: maculopapular CM (which is further subdivided into urticaria pigmentosa, telangiectasia macularis eruptiva perstans and papular/plaque variant), diffuse CM (DCM), and solitary mastocytoma.[4]

Although mastocytosis can occur at any age, the disorder mostly occurs in two age groups: pediatric-onset mastocytosis and adult-onset mastocytosis, which may differ in their clinical manifestations and disease course.

About two-thirds of all CMs occur in children,[1] Urticaria pigmentosa and mastocytomas being the most common types.[5],[6] Pseudoxanthomatous mastocytosis is a rare variety of DCM and is characterized by the presence of yellowish papules or nodules lesions.[4],[7] There is a paucity of case reports of the psuedoxanthomatous variant from India. Herein, we report one such case of a localized form of pseudoxanthomatous mastocytosis, in a 10-year-old girl.

  Case Report Top

A 10-year-old female child presented with asymptomatic yellow colored raised lesions over genitalia for 6 months, which had been growing in size and number. On physical examination, multiple firm, round to oval, skin to yellow colored papules and nodules varying in size from a few millimeters to 1–2 cm in diameter, over labia majora and fourchette [Figure 1]. On rubbing the lesion, there was no erythema or urtication (Darrier sign negative). No other body sites were involved.
Figure 1: Multiple firm, round to oval, skin to yellow colored papules and nodules

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No history of systemic complaints such as flushing, dizziness, palpitation, pain abdomen, bone pain, diarrhea, fever, or weight loss was given. Any possibility of sexual abuse was also ruled out by taking history. The general physical and systemic examination was otherwise normal.

Differential diagnosis of xanthoma, Juvenile xanthogranuloma, mastocytosis, and benign cephalic histiocytosis was made.

Routine investigations such as complete blood count, peripheral smear, liver, and kidney function tests (liver function test and renal function test) and serum lipid profile were within the normal limits. A blood examination, including serum tryptase and serum lipid levels, were within the normal ranges.

A punch biopsy was done from a representative lesion, and histopathology revealed dense diffuse infiltrate of mast cells in the form of uniform round cells with eosinophilic granular cytoplasm in the interstitium of the reticular dermis [Figure 2] Giemsa stain was positive for mast cells. [Figure 3] shows numerous mast cells and eosinophils in deeper dermis.
Figure 2: Infiltration of mast cells in the interstitium of the reticular dermis (H and E, 10x)

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Figure 3: Numerous mast cells and eosinophils (H and E, ×40)

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On correlating the clinical and histopathological examination, a diagnosis of psuedoxanthomatous mastocytosis was made.

She was prescribed oral ketotifen at the dose of 1 mg/day and levocetirizine 2.5 mg daily, along with an emollient. Counseling was done to avoid triggering factors.

[Figure 4] shows the regression of cutaneous lesions after 2 months. The patient is on regular follow-up.
Figure 4: Regression of cutaneous lesions after 2 months

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  Discussion Top

Mastocytosis is a proliferative disorder of mast cells with a wide clinical spectrum, ranging from only cutaneous lesions to involvement of various internal organs suh as lymph nodes, spleen, liver, and bone marrow. This group of the disorder is characterized by mast cells aggregate around blood vessels, and are sometimes associated with eosinophils.[8] The numbers of mast cells can be as high as ten times over normal skin.[9]

Up to 65% of mastocytosis are found in children, UP and mastocytomas being the most common types.[5],[6],[10]

DCM is a rare form of CM that accounts for 1%–3% of CM.[11] As compared to other forms of CM, DCM is more likely to be associated with a more severe form of systemic symptoms such as hypotension, shock, and diarrhea due to a higher concentration of mast cells.[12] These systemic symptoms are due to the release of mast cell mediators such as histamine, heparin, tryptase, chymase, proteases, leukotrienes, prostaglandins, platelet-activating factor, and others.

In contrast to adult-onset mastocytosis, the disease in the pediatric population is usually confined to the skin and is benign and resolves spontaneously.[11] In most children, i.e., around 90% of cases, systemic involvement is absent, just as in our case.

Darier's sign was negative in our case. Although Darier's sign is highly specific of mastocytosis, it may not be positive in all cases, and positivity ranges from 88% to 92%.[10]

Pseudoxanthomatous mastocytosis is an extremely rare variant presenting as homogeneous yellow or cream-colored papules and nodules of varying sizes. The term “xanthelasmoid mastocytosis” (“xanthelasmoidea”) was coined by Fox in 1875.[13]

According to the clinical data published to date, the terms “xanthelasmoid,” “nodular” and “pseudoxanthomatous” mastocytosis describe all the same clinical conditions and therefore, can be considered as synonyms.[7]

Although the exact prevalence of the disease is not clear, based on a previous study, it was observed in 10 of 280 mastocytosis patients.[4]

The lesions can be localized or disseminated, without the classic Darier's sign positivity.

In the present case and in the cases reported by by Pérez-Pérez et al.[7] and Li et al.,[14] the lesions occurred in children on the vulva; thus, we can confer that pseudoxanthomatous mastocytosis is more common in children, and vulva might be a site of predilection in localized cases.

Skin biopsy is the gold standard in the diagnosis of this condition and helps us to rule out other mimickers such as xanthoma and histiocytosis. Treatment includes antihistaminics with or without mast cell stabilizers.

Our case responded very well to oral levocetirizine and ketotifen. As most of the childhood, CM is benign, it is important to counsel the parents regarding the self-resolving yet chronic nature of this condition.

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


Conflicts of interest

There are no conflicts of interest.

  References Top

Horny HP, Sotlar K, Valent P, Hartmann K. Mastocytosis: A disease of the hematopoietic stem cell. Dtsch Arztebl Int 2008;105:686-92.  Back to cited text no. 1
Nettleship J, Tay W. Rare forms of urticaria. Br Med J 1869;2:323-4.  Back to cited text no. 2
Horny HP, Akin C, Metcalfe DD, Escribano L, Bennett JM, Valent P, et al. Mastocytosis (mast cell disease). In: Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, et al., editors. World Health Organization (WHO) Classification of Tumours. Pathology & Genetics. Tumours of Haematopoietic and Lymphoid Tissues. Lyon, France: IARC Press; 2008. p. 54-63.  Back to cited text no. 3
Lange M, Niedoszytko M, Nedoszytko B, Łata J, Trzeciak M, Biernat W. Diffuse cutaneous mastocytosis: Analysis of 10 cases and a brief review of the literature. J Eur Acad Dermatol Venereol 2012;26:1565-71.  Back to cited text no. 4
Inamadar AC, Palit A. Cutaneous mastocytosis: Report of six cases. Indian J Dermatol Venereol Leprol 2006;72:50-3.  Back to cited text no. 5
[PUBMED]  [Full text]  
Ritambhra, Mohan H, Tahlan A. Urticaria pigmentosa. Indian J Dermatol Venereol Leprol 2001;67:33-4.  Back to cited text no. 6
Pérez-Pérez L, Allegue F, Caeiro JL, Fabeiro JM, Pérez Rodríguez A, Zulaica A. Coexistence of two types of clinical lesions in childhood-onset mastocytosis. Indian J Dermatol Venereol Leprol 2011;77:184-7.  Back to cited text no. 7
Kasper CS, Tharp MD. Quantification of cutaneous mast cells using morphometric point counting and a conjugated avidin stain. J Am Acad Dermatol 1987;16:326-31.  Back to cited text no. 8
Garriga MM, Friedman MM, Metcalfe DD. A survey of the number and distribution of mast cells in the skin of patients with mast cell disorders. J Allergy Clin Immunol 1988;82:425-32.  Back to cited text no. 9
Kiszewski AE, Durán-Mckinster C, Orozco-Covarrubias L, Gutiérrez-Castrellón P, Ruiz-Maldonado R. Cutaneous mastocytosis in children: A clinical analysis of 71 cases. J Eur Acad Dermatol Venereol 2004;18:285-90.  Back to cited text no. 10
Srinivas SM, Dhar S, Parikh D. Mastocytosis in children. Indian J Paediatr Dermatol 2015;16:5763.  Back to cited text no. 11
  [Full text]  
Wolff K, Komar M, Petzelbauer P. Clinical and histopathological aspects of cutaneous mastocytosis. Leuk Res 2001;25:519-28.  Back to cited text no. 12
Fox T. On xanthelasmoidea (an undescribed eruption). Trans Clin Soc London 1875;8:53-7.  Back to cited text no. 13
Li Q, Zeng K, Peng X, Wang F. Dermoscopic findings of pseudoxanthomatous mastocytosis localized on vulva. An Bras Dermatol 2018;93:940-1.  Back to cited text no. 14


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


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