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A m e R I c a N A c a D e m Y OF

D e r M a T O L O G Y IIIIII Ill II IIIII II II I

Answers for Self-Assessment examination of the American Academy of Dermatology

Identification No. 882-209 September, 1982, issue of the JOURNAL OF THE AMERICAN A C A D E M Y O F D E R M A T O L O G Y

QUESTIONS 1-4

Chronic bullous dermatosis of childhood, or linear IgA dermatosis of childhood, can only be diagnosed on the basis of the immunofluorescent pattern. The clinical picture of blisters arranged in a rosette pattern in the groin has been described in lesions of bullous pemphigoid and childhood der- matitis herpetiformis as well as linear IgA der- matosis. Also, the presence of a subepidermal blister is not specific, although it does rule out pernphigus.

Vitamin A is not recommended for treatment. All the other approaches are reasonable, but Jab- lonska has endorsed the combination of systemic corticosteroids and dapsone as most effective. The disease is likely to be chronic, but none of the other associations suggested have been docu- mented.

The recommended choices for this series are: i, a, c; 2, a, d, e; 3, a; 4, a.

REFERENCE 1. Chorzelski TP, Jablonska S: IgA linear dermatosis of

childhood (chronic bullous disease of childhood). Br J Dermatol 101:535-542, 1979.

QUESTIONS 5-10

The clinical appearance in this case most strongly suggests lichen nitidus, since the papules are uni- formly small, flat, and nonpruritic. Their location and linearity on the hand should make one con- sider verruca plana, rather than keratosis pilaris since the latter is usually present on the cheeks, posterior arms, and anterior and posterior thighs. In addition, early lesions of lichen planus may be similarly small and pinheadlike. Lichen striatus would not be seriously considered, since it is

abrupt in onset, confluent, and usually in a linear distribution on an extremity.

Other typical locations of lichen nitidus include genitalia (especially in males), trunk, breasts, but- tocks, and occasionally oral mucosa. Lesions of the scalp have not been noted.

The biopsy presented is characteristic of lichen nitidus. A central parakeratotic cap is seen, in contrast to the orthokeratosis of lichen planus. The epidermis seems to embrace the inflammatory infiltrate, which is fundamentally lichenoid, but is also granulomatous and contains multinucleated giant cells. Epidermal basal cell layer damage oc- curs, and there is incontinence of pigment, but immunoglobutins are absent. The reaction in kera- tosis pilaris would be centered on a hair follicle. Verruca plana and lichen striatus show charac- teristic epidermal changes of prickle cell edema and dyskeratosis, respectively.

Of the diseases listed as possibly associated with lichen nitidus, only lichen planus is accepted. Even this allegation has led to dispute about pos- sible confusion with early lesions of lichen planus. Multiple histologic sections and continued obser- vation of the patient may be needed to make the distinction.

No therapy is generally regarded as effective, or indeed even necessary, in most cases, since the disease is usually asymptomatic. The usual out- come of the lesions is slow resolution, with no lasting pigmentary change or atrophy.

For this series the recommended choices are: 5, b, c, d; 6, a, b, c, d; 7, c; 8, d; 9, e; 10, a.

REFERENCES 1. Hurley H J: Papulosquamous eruptions and exfoliative

dermatitis, in Moschella SL, et al, editors: Dermatology.

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Volume 7 Number 3 September, 1982

Philadelphia, 1975, W. B. Saunders Co., pp. 440-443. 2. F'inkus H, Mehregan AH: A guide to dermatohistopathol-

ogy, ed. 2. New York, 1976, Appleton-Century-Crofts, pp. 150-152,203,429.

QUESTIONS 11-14

The lesions on this patient identify his eruption as papulosquamous. But because the eruption is so recent, distinguishing clinical features are difficult to find. However, one would expect them to be more nearly oval in pityriasis rosea, and to exhibit some purpura in pityriasis lichenoides chronica. Secondary syphilis, guttate psoriasis, and drug eruption would all be serious considerations.

Each of the historical and physical points listed under the second stem would be pertinent, but of varying degrees of importance. All medications taken should be considered, especially since a papulosquamous eruption would be an unusual penicillin reaction. The preceding episode of pharyngitis is clearly of potential importance, es- pecially if its cause could be identified as strep- tococcal. Streptococcal pharyngitis is a frequent antecedent of guttate psoriasis. Fever, sore throat, and other constitutional symptoms could relate either to streptococcal pharyngitis or to syphilis. Involvement of palms and soles would strongly favor secondary syphilis, as would generalized lymphadenopathy. A family history of psoria- sis would support that diagnosis, but its ab- sence would certainly not make psoriasis less likely.

Considering all of the above, the best clinical diagnosis is guttate psoriasis. Secondary syphilis could be ruled in or out with the VDRL. Routine histopathology could be helpful also. Although the fully developed histologic picture of psoriasis might not appear in lesions of only 9 days' dura- tion, typical epidermal changes would be expected; i.e., slight psoriasiform hyperplasia with subcor- neal and intracorneal neutrophilic pustules. Immu- nofluorescence and electron microscopy would have no specific patterns, and intradermal peni- cillin tests would probably not be helpful in con- sideration of a papular exanthematous type of eruption.

The recommended choices for this series are: 1l, a, b, e; 12, a, b, c, d, e; 13, b; 14, a, c.

Self-Assessment examination answers 47A

REFERENCES 1. Baker H, Wilkinson DS: Psoriasis, in Rook A, et al,

editors: Textbook of dermatology, ed. 3. Oxford, 1979, Blackwetl Scientific Publications, pp. 1326-1327.

2. Ackerman AB: Histologic diagnosis of inflammatory skin diseases. Philadelphia, 1978, Lea & Febiger, p. 252.

QUESTIONS 15-18

Angioendotheliomatosis is a rare condition hav- ing two separate clinical presentations: a reactive, benign type that is associated with subacute bac- terial endocarditis, and a "malignant" type with fulminant spread and death. Fever is common in both types, and the histopathology is similar in both, with occlusion of vascular lumina by pro- liferating and swollen endothelial cells. The same histopathologic changes can be seen in cutaneous lesions of subacute bacterial endocarditis (Osler's nodes, Janeway's lesions, petechial hemorrhages), circulating immune complexes, cholesterol era- boll, and bacterial sepsis. The clinical course of reactive endotheliornatosis, however, is benign, while patients with the "'malignant" type often have vascular luminal involvement of kidneys, heart, lungs, brain, or gastrointestinal tract. No internal malignancy has been reported in associa- tion with angioendotheliomatosis.

Recommended choices for this series are: 15, a, b, c, d; 16, a, b, c, d, e; 17, a, b, c, d, e; 18, e.

REFERENCE 1. Martin S, Pitcher D, Tschen J, Wolf JE Jr: Reactive an-

gioendotheliomatosis. J AM ACAD DERMATOL 2:117-123, 1980.

QUESTIONS 19-24 Polymorphous light eruption (PMLE) is a dis-

tinct disease entity characterized by the sudden onset of pruritic lesions, usually localized to one area of exposed skin which the patient can easily recall. There is subsequent spread to other ex- posed skin surfaces.

PMLE is generally classified according to types of lesions and length of sun exposure that can be tolerated. The most severe is the erythematous type. These patients generally cannot tolerate more than 15 minutes of outdoor mid-summer, mid-day, temperate zone sunlight. Other types (papular and plaque) are less sensitive, and in

Volume 7 Number 3 September, 1982

Self-Assessment examination answers 51A

some instances may tolerate up to 1 hour or more of sun exposure. These eruptions are inde- pendent of skin type.

Although the histopathology may be similar to discoid lupus erythematosus, immunofluorescence studies and antinuclear antibodies are negative in PMLE. Phototesting is the only means of repro- ducing the disease. With either a monochromator or appropriate filters and artificial light of a con- tinuous spectral distribution, the precise action spectrum can be determined.

A frequent occurrence that may go unnoticed in these patients is cheilitis. Photophobia, malaise, headaches, and other constitutional symptoms fre- quently occur when patients are exposed to sun- light for prolonged periods.

This disease is characterized by its uniqueness of becoming less intense (within weeks to 2 months) as more light exposure is received during the spring and/or summer months (hardening.phe- nomenon). Other photosensitivity disorders in the differential diagnosis tend to worsen with increas- ing light exposure.

Many cases of PMLE undergo spontaneous resolution. Other photosensitivity disorders con- sidered in the differentiaI diagnosis will not do so.

For this series the recommended choices are: 19, c; 20, a, b, d; 21, c; 22, b, d; 23, a; 24, d.

REFERENCES

1. Magnus IA: Studies with a monochromator in the common idiopathic photodermatoses. Br J Dermatol 76:245-264, 1964.

2. Brothagen H: Polymorphous light eruptions, in Fitzpatrick TB, editor: Sunlight and man. Tokyo, 1976, Tokyo Uni- versity Press, pp. 679-693.

3. Jansen CT: The natural history of polymorphous light eruptions. Arch Dermatol 115:165-169, 1979.

QUESTIONS 25-27

In most cases the eruption of congenital cutane- ous candidiasis is noted at birth or within 12 hours after delivery. Lesions are scattered diffusely over the trunk, neck, head, and the nails; palms and soles also have been involved. Oral lesions usually are absent and the diaper area is spared, Inter- triginous areas, the back, and extensor surfaces of

the extremities may be severely affected. Indi- vidual lesions begin as intensely erythematous macules and papulovesicles that become pustular. The lesions dry within 4 to 7 days, producing a prominent postinflammatory desquamation.

The cutaneous infection is characterized by aa absence of constitutional signs and uncomplicated recovery is the rule. The WBC and platelet counts have been normal, as have the total lymphocyte count and lymphocyte stimulation studies with Candida albicans and phytohemagglutinin.

Congenital cutaneous candidiasis does not ap- pear to be related to maternal age, parity, type of delivery, duration of labor, or gestational age. Moreover, in contrast with neonatal bacterial in- fection, congenital cutaneous candidiasis does not appear to be related to duration of rupture of membranes. Candida cannot pass the placental barrier and, therefore, ascending infection is felt to be the route of invasion.

The clinical features of congenital cutaneous candidiasis are usually distinctive enough to per- mit differentiation from other dermatoses that occur during the first few days of life. Those con- ditions in the differential diagnosis include ac- ropustulosis of infancy, transient neonatal pustular melanosis, and erythema toxicum. The diagnosis of congenital cutaneous candidiasis is easily confirmed by microscopic examination of KOH- treated scrapings of skin or culture of scrapings on Sabouraud's medium.

Impetigo of the newborn commonly develops during the first week of Iife. Large bullae contain- ing fluid, which is at first clear and later may be purulent, develop on the face, trunk, and limbs. The morphology of these lesions differs from those of congenital cutaneous candidiasis.

For this series the recommended choices are: 25, a; 26, d; 27, c.

REFERENCES

l. Kam LA, Diacoia GP: Congenital cutaneous candidiasis. Am J Dis Child 129:1215-1218, 1975.

2. Rudolph N, Tariq AA, Reale MR, et al: Congenital cuta- neous candidiasis. Arch Dermatol 113: 1101-1103, 1977.