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INTRODUCTION

This chapter highlights several benign proliferative disorders seen in the skin such as cutaneous mastocytosis, cutaneous histiocytosis, cutaneous plasmacytosis, and inflammatory pseudotumor.

CUTANEOUS MASTOCYTOSIS

The various types of cutaneous mastocytosis may be considered as variations on a theme which centers on mutations of the cell surface tyrosine kinase, c-kit receptor (Figures 13–1, 13–2, 13–3, 13–4, 13–5, 13–6, 13–7, 13–8, 13–9, 13–10, 13–11, 13–12, 13–13, 13–14). The spectrum of clinical presentation ranges from telangiectactic macules seen in telangiectasia macularis eruptiva perstans, through pigmented macules that urticate when rubbed as seen in urticaria pigmentosa, to maculopapular mastocytosis and nodular mastocytosis.

Figure 13-1

Telangiectasia macularis eruptiva perstans

Telangiectasia macularis eruptiva perstans (TMEP) is an uncommon form of cutaneous mastocytosis, which occurs more frequently in adults. Lesions are typically brown to erythematous macules with overlying telangiectasia located on the anterior trunk and upper limbs.

Figure 13-2

Telangiectasia macularis eruptiva perstans

Multiple scattered tan macules are present on the trunk in the same patient. Lesions usually do not urticate on rubbing. Systemic involvement of the bone marrow, gastrointestinal system, hepatobiliary system, and lymph nodes occurs very infrequently.

Figure 13-3

Telangiectasia macularis eruptiva perstans—close-up

Reticulate matted telangiectasias on the trunk in TMEP.

Figure 13-4

Urticaria pigmentosa—child

This is the most common form of cutaneous mastocytosis. It usually develops in the first year of life and presents as multiple reddish-brown macules, papules, plaques, or nodules with the highest concentration on the trunk and thighs. Darier’s sign is positive. Systemic involvement is uncommon in children as compared to adults.

Figure 13-5

Urticaria pigmentosa—adult

Urticaria pigmentosa can occasionally persist till adulthood or develop de novo in adulthood. The prognosis for urticaria pigmentosa in adults is less favorable with a more chronic and protracted course. Patients are also more likely to have systemic involvement with symptoms of excessive mediator release (e.g., flushing, headaches, vascular instability, peptic ulceration and diarrhea) and systemic involvement (e.g., bone pain).

Figure 13-6

Urticaria pigmentosa—close-up

Disseminated red macules in urticaria pigmentosa.

Figure 13-7

Maculopapular mastocytosis

Extensive maculopapular mastocytosis with widespread erythematous papules on the trunk. Patients with maculopapular mastocytosis usually have indolent systemic mastocytosis with a good prognosis.

Figure 13-8

Maculopapular mastocytosis

Reddish-brown papules and nodules coalescing on the back. Investigations including full blood counts, serum tryptase, skeletal survey, bone densitometry, ...

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