Cutaneous mastocytoma Q82.2

Author: Prof. Dr. med. Peter Altmeyer

Co-Autor: Max Zhao

All authors of this article

Last updated on: 29.10.2020

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Synonym(s)

cutaneous mastocytoma; Mast cell nevus; solitary mastocytoma

History
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Fox, 1875

Definition
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After Urticaria pigmentosa, the second most common manifestation form of cutaneous mastocytosis with localized, nodular, cutaneous mast cell proliferation, clinically manifested as yellowish-brown, 1-10cm large, sharply defined plaques or nodules.

Manifestation
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Occurs from birth or in the first months of life (90% of mastocytomas occur within the first two years of life).

Of all childhood mastocytoses, 20% occur as mastocytomas.

m:f=1:4

Localization
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Often located on the trunk, less frequently on the face or extremities.

Clinical features
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Solitaires or a few, rarely multiple, 0.5-5.0 cm large, rarely larger (up to 10 cm in size), firm, brownish-violet or brownish-yellow, callous, raised, painless nodules or plaques with a smooth (never scaly) surface.

The surface of mastocytomas is not smooth atrophic but shows either the normal relief of the field skin as in the surrounding area, or a slightly coarsened (lichenified) pattern. This morphological sign is of diagnostic importance for differentiation from other proliferative processes (e.g. lymphoma of the skin). After rubbing the foci or after taking codeine-containing medication (Siebenhaar F et al. 2012), strong urticarial (rarely even bullous) reactions ( Darian's sign), possibly combined with itching, can be triggered. Warming also leads to a reactive redness (this is usually observed by parents when their children take warm baths).

A rare subtype of childhood mastocytosis is diffuse cutaneous mastocytosis, which becomes evident in the first weeks of life. It is characterized by a generalized, sulfurous thickening of the skin. Mechanical irritation can trigger urticarial dermographism.

Histology
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The surface epithelium is unremarkable. Usually the entire dermis is permeated by a diffuse or nodular infiltrate of mast cells, which can extend into the subcutis. The cell type occurring in mastocytomas is monomorphic, oval or polygonal with a weakly eosinophilic cytoplasm and oval uniform nuclei. Eosinophils may be added to the mast cell aggregates in varying densities. The aggregates can be detected by means of monoclonal antibodies against the SCF receptor (stem cell factor - CD117).

Differential diagnosis
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  • Clinical differential diagnoses:
    • Xanthogranuloma: mostly isolated, rarely multiple, colour rich yellow, surface either roughly grooved or smooth; no Darian sign!
    • Granuloma glutaeale infantum: in typical localization on the buttocks in the diaper area; color brown; rough surface; no Dariere's sign.
    • Lymphadenosis cutis benigna (nodular form): predominant on the capillitium, appearing as asymptomatic, firm, red, brown to brown-red papules or nodules. Mostly occurring beyond the age of 1 year. No Darian sign.
    • Benige's cephalic histiocytosis: brownish, flat, firm papules on the sceleton and trunk. Darian sign negative.
    • Spitz-Naevus: solitary, reddish or reddish-brown, firm papules, Dari characters negative.
  • Histological differential diagnosis:
    • Histological DD are juvenile xanthogranulomas, Langerhans cell histiocytosis, melanocytic nevi and B-cell type lymphomas. Inconspicuous epidermis, the diffuse monomorphic cell infiltration of the dermis, the typical cells with their pale eosinophilic cytoplasm, frequent histoeosinophilia, the typical histochemical (Giemsa: metachromasia) and immunohistological behaviour define the mastocytoma beyond doubt.

Therapy
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As a rule, no therapy is required; almost always spontaneous healing without complications. In case of itching application of a local antihistamine such as clemastine (e.g. Tavegil Gel). In the absence of regression or if the symptoms are pronounced, an excision can be considered (a rare option!).

External therapy
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Good effects were achieved by local tacrolimus applications.

Progression/forecast
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Regular regression within months to years until adolescence. S.a. Mastocytosis.

Note(s)
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In cutaneous juvenile mastocytosis the clear distinction between mastocytomas and urticaria pigmentosa becomes blurred. Thus, by definition, the diagnosis of mastocytoma(s) is made up to the number of 5 lesions. With > 5 "mastocytomas" the diagnosis is "Urticariapigmentosa". This distinction is not necessary in adult cutaneous mastocytosis, as the clinical picture is very different from that of adult mastocytosis (disseminated, rather small foci, very numerous foci, colour: rather deep brown and red-brown). Cf. the illustration. The "adult cutaneous mastocytoma" does not seem to exist in the type known in children!

Literature
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  1. Fox WT (1875) On xanthelasmoidea (an undescribed eruption). Trans Clin Soc London 8: 53
  2. Frieri M et al (2013) Pediatric Mastocytosis: A Review of the Literature. Pediatric Allergy Immunol Pulmonol 26:175-180

  3. Kang NG et al (2002) Solitary mastocytoma improved by intralesional injections of steroid. J Dermatol 29: 536-538
  4. Méni C et al (2015) Paediatric mastocytosis: a systematic review of 1747 cases. Br J Dermatol 172:642-651
  5. Shuangshoti S et al (2003) Solitary mastocytoma of the vulva: report of a case. Int J Gynecol Pathol 22: 401-403
  6. Siebenhaar F et al (2012) Chilldhood onset mastocytosis. Dermatologist 63: 104-109
  7. Sukesh MS et al (2014) Solitary mastocytoma treated successfully with topical tacrolimus. F1000Res 3:181

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Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 29.10.2020