Slideshow
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October 2015 Dermatology Clinic
A healthy 17-year-old white male presents to the emergency department with mild pruritis and a maculopapular rash on his bilateral upper and lower extremities and beneath the waistband of his swimsuit that he notes had developed while swimming in the Atlantic Ocean. He reports having experienced these same symptoms intermittently over the years when on vacation from his home in Maine; these symptoms were treated successfully with an H2 blocker and antihistamines. The patient denies shortness of breath, dysphagia, or cough. No wheezing or facial or oral swelling are noted. Review of systems is negative. The patient’s medical history is unremarkable, and he does not take any medications. He has a food allergy to chicken.
This The Clinical Advisor CME activity consists of 3 articles. To obtain credit, read Pink, scaly rash on the trunk and extremities and Scaling rash on the palms. Then take the post-test here.
Submit your diagnosis to see full explanation.
Mastocytosis refers to a group of disorders characterized by excessive mast cell accumulation in one or multiple tissues.1 Mast cells originate from the bone marrow progenitor cells and are distributed throughout the connective tissues. They are concentrated in the skin around the peripheral nerves and adjacent to blood and lymphatic vessels.2
The mastocytoses comprise a heterogeneous group of disorders that demonstrate mast cell proliferation in the skin and/or other tissues.3 The most common organ affected is the skin. It can be further classified as being of childhood onset (less than age 15 years) or adult onset (more than age 15 years).4
CM is divided into 3 groups: maculopapular-type CM (also known as urticaria pigmentosa), diffuse CM, and solitary mastocytoma.1 Systemic mastocytosis describes forms of mastocytosis in which pathologic mast cells infiltrate multiple extracutaneous organs, with or without skin involvement.1 The most common presentation of CM is maculopapular, in which mast cell infiltration is limited to the skin, in the absence of involvement of other organs.1 This form will be presented here.
The course of maculopapular-type CM is usually transient and benign in nature. A family predisposition occurs in rare cases. Approximately 2/3 of patients affected are children.5 Most improve or completely resolve by adolescence. Adults more frequently present with systemic forms and have persistent disease.
Symptoms are generally initiated through the mechanical irritation of skin lesions (often by rubbing), which leads to a release of mast cell mediators. This release of mast cell mediators (within about 5 minutes) causes the reddening (flushing) and urticarial swelling that is typical in all forms of CM. This reaction is known as Darier’s sign.5 This effect occurs when physical irritation triggers the localized release of mast cell mediators. The characteristic lesions of CM, which are present in most patients, are called urticaria pigmentosa and are small, yellow-tan-to-reddish-brown macules or slightly raised papules. These lesions are the most common finding in both cutaneous and systemic mastocytosis.
The upper and lower extremities are the most commonly affected areas, followed by the trunk and abdomen. These areas are affected in 70% to 90% of cases of mastocytosis in children.6 Pruritis associated with urticaria pigmentosa may be exaggerated by extremes or sudden changes in temperature, exercise, hot showers, local friction, ingestion of hot beverages, spicy foods, stress, and some medications.1
The differential diagnoses related to rashes are vast and must be focused based on information elicited through history taking. Two possibilities specific to CM include erythema multiforme and pityriasis rosea. Like CM, erythema multiforme minor is an acute, self-limiting, inflammatory skin eruption considered to be a hypersensitivity reaction that may cause minor burning and/or itch. Unlike CM, erythema multiforme minor will usually last 3 to 5 weeks before receding, does not involve the mucous membranes, is thought to be autoimmune in nature, and usually follows infection (ie, herpes simplex, mycoplasma, fungal disease) or drug exposure (ie, barbiturates, penicillins, phenytoin, sulfonamides).7 Pityriasis rosea is a benign papulosquamous eruption that has a duration of 6 to 8 weeks and affects primarily older children and young adults. It is usually noninfectious and self-limiting, evolves rapidly, and often begins with a single patch (herald) that later is followed by smaller patches. CM presents as a maculopapular rash that is not preceded by a herald patch and that causes pruritis and erythema when stroked (Darier’s sign).5
Diagnosis of CM is generally based on clinical presentation and histologic findings of increased numbers of mast cells.3 Obtaining a thorough history aids in accurate diagnosis as well. The SCORMA (SCORing MAstocytosis) Index is used to evaluate the severity of CM.8 The SCORMA index combines the scores of 3 parts: The first part (a) is the assessment of skin involvement, the second part (b) is an estimation of the activity of the lesions, and lastly, the third part (c) is a recording of the subjective symptoms.9 Serum tryptase is also used in detecting disease severity.
More than 80% of children with CM will experience spontaneous resolution, whereas the remaining children may have persistent symptoms and may possibly progress to systemic forms of this disease.6 Patients with CM may have concomitant immunoglobulin E (IgE)-mediated allergic diseases, including allergic rhinitis, food and drug allergies, and hymenoptera allergy. They may also have asthma.
Treatment varies based on severity of symptoms. Frequently affected individuals recover spontaneously and rarely require treatment. The first step in treatment is to avoid triggering factors that can produce a release of mast cell mediators; these triggers are specific for each patient. Pharmacologic management consists of medications to prevent release of mast cell mediators and treatment to decrease symptoms when a mediator release does occur. H1 antihistamines are useful in preventing flushing and itching. H2 antihistamines may enhance the antipruritic effects of H1 antihistamines. Commonly used H1 antihistamines include oral cetirizine (10 mg daily), fexofenadine (180 mg daily), or hydroxyzine (25 mg every 6 hours); each of these medications may be used in children at doses appropriate to age and weight.1 H2 antihistamines include oral ranitidine (150 mg every 12 hours), famotidine (10 mg every 12 hours), and cimetidine (400 mg twice daily); ranitidine and famotidine may be used in children at doses appropriate to age and weight.6 The use of glucocorticoids decreases inflammation through inhibiting further histamine release.
The patient in this case was treated with diphenhydramine (50 mg by mouth), famotidine (40 mg by mouth), and prednisone (60 mg by mouth) in the emergency department with resolution of approximately 80% of symptoms obtained before discharge. His itching and discomfort subsided substantially. He was discharged with prescriptions for prednisone (60 mg by mouth daily for 5 days), famotidine (40 mg by mouth daily), and diphenhydramine (50 mg by mouth every 6 hours as needed for itching). The patient was also instructed to keep a diary on the circumstances before and when the rash occurs and to follow up with a dermatologist when he returned home to Maine.
Jean Holden, MSN, FNP-BC, is an advanced practice nurse at Urgent Care Now in Lacey, N.J., and Kristen Patterson, MSN, RN, CSN, is a certified school nurse in the Lacey Township School District in Forked River, N.J., and an adjunct professor and PhD student in Nursing Education at Kean University in Union, N.J.
This The Clinical Advisor CME activity consists of 3 articles. To obtain credit, read Pink, scaly rash on the trunk and extremities and Scaling rash on the palms. Then take the post-test here.
References
- Castells MC and Akin C. Mastocytosis (cutaneous and systemic): Epidemiology, pathogenesis, and clinical manifestations. Updated June 23, 2015. Available at uptodate.com/contents/mastocytosis-cutaneous-and-systemic-epidemiology-pathogenesis-and-clinical-manifestations
- Alto WA, Clarcq L. Cutaneous and systemic manifestation of mastocytosis. Am Fam Physician. 1999;59(11):3047-3054, 3059-3060.
- Weinberg JM. How to diagnose and treat mastocytosis. The Clinical Advisor. 2008;11(3):43-47. Available at clinicaladvisor.com/features/how-to-diagnose-and-treat-mastocytosis/article/117770
- Güneş Bilgili SG, Karadağ AS, Takci Z, Çalka Ö, Kösem M. Comparison of cutaneous mastosytosis with onset in children and adults. Turk J Med Sci. 2014;44(3):504-510.
- Lange M, Niedoszytko M, Renke J, Gleń J, Nedoszytko B. Clinical aspects of paediatric mastocytosis: A review of 101 cases. J Eur Acad Dermatol Venereol. 2013;27(1):97-102.
- Castells M, Metcalfe DD, Escribano L. Diagnosis and treatment of cutaneous mastocytosis in children: Practical recommendations. Am J Clin Dermatol. 2011;12(4):259-270.
- Murphy-Lavoie H, LeGros TL. Emergent diagnosis of the unknown rash: An algorithmic approach. Emergency Medicine. 2010;42(3):6-17.
- Heide R, Middelkamp Hup MA, Mulder PG, Oranje AP; Mastocytosis Study Group Rotterdam. Clinical Scoring of Cutaneous Mastocytosis. Acta Derm Venereol. 2001;81(4):273-276.
- Lange M, Niedoszytko M, Renke J, Gleń J, Nedoszytko B. Clinical aspects of paediatric mastocytosis: A review of 101 cases. J Eur Acad Dermatol Venereol. 2013;27(1):97-102.
All electronic documents accessed on October 2, 2015.
This The Clinical Advisor CME activity consists of 3 articles. To obtain credit, read Pink, scaly rash on the trunk and extremities and Scaling rash on the palms. Then take the post-test here.
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