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Congenital Dermal Melanocytosis

FIGURE 17-17. Nevus of Ota. (Copyright 2015 by the American Academy of Dermatology.

All rights reserved.)

II. Therapeutic Modalities

A. Camouflage is the usual approach to treatment of the condition.

FIGURE 17-18. Congenital dermal melanocytosis. (Copyright 2015 by the American Academy of Dermatology. All rights reserved.)

II. Therapeutic Modalities

A. No specific treatment is required.

B. The color reaches a peak at 1 to 2 years of age and then begins to fade.

C. The majority of lesions are absent by adolescence.

D. Occasionally, lesions persist to adulthood and are usually localized to the buttocks.

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STUDY QUESTIONS

1. Piebaldism is an acquired disease of hypopigmentation.

a. True b. False

2. Albinism is a disorder of hypopigmentation secondary to a defect in which of the following?

a. Melanocyte b. Keratinocyte c. Lymphocyte d. Melanophage

3. Which of the following statements regarding melasma is false?

a. There is increased number and activity of melanocytes.

b. Hormones may influence the condition.

c. It is most commonly treated with narrowband UVB.

d. Common distribution is on the face.

4. Hyperpigmentation of the skin may be caused by which of the following?

a. Antimalarials b. Heavy metals c. Infection

d. Topical tar-containing preparations e. A, B, and C

f. A and C

g. All of the above

5. First-line treatment for Becker nevus is surgical excision.

a. True b. False

6. Nevus spilus commonly evolves into melanoma.

a. True b. False

7. Congenital dermal melanocytosis occurs rarely on the buttocks of Asian newborns.

a. True b. False

8. Which of the following comprise the follicular occlusion triad?

a. Psoriasis, hidradenitis, and acne b. Hidradenitis suppurativa

c. Acne conglobata d. Dissecting cellulitis e. B, C, and D

9. Dermatosis papulosa nigra is:

a. Most unique to people of African descent b. A benign condition

c. Commonly seen as numerous 1- to 5-mm papules d. Cosmetically unacceptable to most individuals e. All of the above

f. A, B, and C

10. Keloids are common in:

a. Dark-skinned individuals

b. Individuals who easily sunburn c. Light-skinned people of Finland d. All of the above

11. The best treatment for pseudofolliculitis is which of the following?

a. Discontinuing shaving the affected area

b. Shaving against the direction of the hair growth c. Applying shaving cream before shaving

d. Shaving as close as possible to the skin surface 12. Longitudinal melanonychia:

a. Reveals melanin deposits in the nail plates b. Are striped in appearance in the nail plates

c. Should include melanoma in the differential diagnosis d. All of the above

e. A and C

13. Clinical features of psoriasis are the same in fair-skinned and dark-skinned individuals.

a. True b. False

Answers to Study Questions

1.b 2.a 3.c 4.g 5.b 6.b 7.b 8.e 9.e 10.a 11.a 12.d 13.b

CHAPTER 18 Hypersensitivities, Drug Eruptions, Vasculitides, and Miscellaneous Inflammatory Disorders

Cathleen K. Case • Kelli Turgeon-Daoust

OBJECTIVES

After studying this chapter, the reader will be able to:

List the four general types of hypersensitivity reactions.

Differentiate between immediate- and delayed-type hypersensitivities.

Understand diseases associated with hypersensitivity reactions.

Define the major characteristics of small-vessel vasculitis.

Understand management of adverse drug reactions.

Describe the spectrum of Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) and identify major features of erythema multiforme (EM).

KEY POINTS

The immune system is designed to protect the host but may fail.

Assessment and history-taking skills are of paramount importance in the evaluation of a suspected hypersensitivity reaction.

Avoidance of allergens and irritants, when possible, is the best treatment of urticaria and other hypersensitivity reactions.

Differential diagnosis of many cutaneous eruptions includes drug reactions.

Urticarial lesions that last longer than 24 hours or present with purpura should be biopsied to rule out urticarial vasculitis.

Episode of urticaria lasting more than 6 weeks is considered chronic urticaria. First-line therapy for urticaria is nonsedating H1 antagonist antihistamines.

Erythema multiforme is a self-limited but potentially recurrent disease, commonly associated with herpes simplex virus infection.

Erythema nodosum is the most common type of panniculitis and may occur in association with systemic disease such as sarcoidosis or inflammatory bowel disease, drug therapy such as oral contraceptive pill, or other infection; half of cases are idiopathic.

Cutaneous small-vessel vasculitis, also called leukocytoclastic vasculitis and hypersensitivity vasculitis, is the most common form of small-vessel necrotizing vasculitis. Palpable purpura is characteristic for this disorder.

The characteristic lesion of pyoderma gangrenosum is a painful ulcer with a rolled or undermined border frequently on the legs; it is neither infectious nor gangrenous.

Toxic epidermal necrosis is a medical emergency with a high mortality rate.

I. OVERVIEW

There are two opposite sides to immunity: host protection and injury. The immune response to an antigen is designed to protect the host. Disorders of the immune system include hypersensitivity, autoimmunity, and immunodeficiency. These response mechanisms produce a wide range of clinical conditions from local reactions to life-threatening diseases. The primary biological effects of the immune response are the humoral (antibody) and cellular recognition and elimination of infectious agents and other foreign antigens. If there is re-exposure to the same antigen in a previously sensitized individual, there may be an exaggerated or

“hypersensitivity” reaction. This is a misdirected immune response that results in local tissue injury or systemic manifestations, which may include shock and death. Immune responses that result in tissue injury or other pathophysiologic changes are called allergic/immunopathologic (hypersensitivity) reactions. There are four types of hypersensitivity reactions.

A. Causes of tissue injury

1. Release of vasoactive substances; primary and secondary mediators 2. Phagocytosis or lysis of cells

3. Activation of components of the complement system

4. Release of proteolytic enzymes, cytokines, and other mediators of tissue injury and inflammation from recruited inflammatory cells

B. Hypersensitivity reactions, their mediators, and associated conditions (Table 18-1)

This chapter focuses on urticaria, angioedema, and anaphylaxis, certain drug reactions, cutaneous small-vessel vasculitis (CSVV), and other hypersensitivity syndromes, including:

1. Erythema multiforme (EM)

2. Stevens-Johnson syndrome (SJS) 3. Toxic epidermal necrolysis (TEN) 4. Erythema nodosum (EN)

5. Pyoderma gangrenosum (PG)

TABLE 18-1 Four Types of Hypersensitivity Reaction