Childhood Solitary Cutaneous Mastocytoma: Clinical Manifestations, Diagnosis, Evaluation, and Management

Abstract

Background: The diagnosis of solitary cutaneous mastocytoma is mainly clinical, based on lesion morphology, the presence of a positive Darier sign, and the absence of systemic involvement. Knowledge of this condition is important so that an accurate diagnosis can be made.

Objective: To familiarize physicians with the clinical manifestations, diagnosis, evaluation, and management of a solitary cutaneous mastocytoma.

Methods: A PubMed search was completed in Clinical Queries using the key term "solitary cutaneous mastocytoma". The search strategy included meta-analyses, randomized controlled trials, clinical trials, observational studies, and reviews. Only papers published in English language were included. The information retrieved from the above search was used in the compilation of the present article.

Results: Typically, a solitary cutaneous mastocytoma presents as an indurated, erythematous, yellow- brown or reddish-brown macule, papule, plaque or nodule, usually measuring up to 5 cm in diameter. The lesion often has a peau d'orange appearance and a leathery or rubbery consistency. A solitary cutaneous mastocytoma may urticate spontaneously or when stroked or rubbed (Darier sign). Organomegaly and lymphadenopathy are characteristically absent. The majority of patients with skin lesions that erupt within the first two years of life have spontaneous resolution of the lesions before puberty. Treatment is mainly symptomatic. Reassurance and avoidance of triggering factors suffice in most cases.

Conclusion: The diagnosis is mainly clinical, based on the morphology of the lesion, the presence of a positive Darier sign, and the absence of systemic involvement. A skin biopsy is usually not necessary unless the diagnosis is in doubt.

Keywords: Darier sign; Indurated; hyperpigmented; macule; nodule; papule; plaque..

Fig. (1)

Increased melanization of the basal layer with dense aggregates of mast cells infiltrating the upper and mid dermis (hematoxylin-eosin stain, original magnification x 40).

Fig. (2)

Mast cells are round or spindled-shaped with abundant eosinophilic cytoplasm and large nuclei. The cytoplasm contains basophilic granules (hematoxylin-eosin stain, original magnification x 400).

Fig. (3)

A solitary cutaneous mastocytoma presenting as a reddish brown plaque on the left posterior thigh of a 4-month-old infant. The lesion had a cobblestone, “peau d’orange” surface.

Fig. (4)

With rubbing, the plaque became urticarial with a surrounding flare of erythema (Darier sign).