Anne Sveen, MD

FigureA 13-year-old developmentally delayed girl was brought for evaluation of fibrotic plaques with overlying atrophy and surrounding hyperpigmentation on the middle back (A), lower right breast (B), and upper arms and thighs. The lesions had appeared 6 to 8 months earlier as purplish patches that "hurt a little." They subsequently progressed to firm, white lesions with an outer rim of violaceous discoloration. Results of a skin biopsy confirmed the clinical diagnosis of localized scleroderma.  

Scleroderma may present at any age and can be localized or systemic. Localized scleroderma affects the skin, subcutaneous fascia, and occasionally muscle and bone. Systemic scleroderma is characterized by chronic disease with both skin changes and visceral abnormalities. Rarely, localized scleroderma may progress to systemic disease; however, screening for this form is unnecessary in patients who have no systemic symptoms.

 Localized scleroderma primarily affects children and young adults. The incidence is 0.4 to 1 per 100,000 persons; the female-to-male ratio is 2:1 to 3:1. In children, the localized form occurs at least 10 times more often than the systemic form.

 Localized scleroderma is subdivided into 2 types:

     Morphea (plaque-type, generalized,     guttate, and profunda).

 This patient had plaque-type morphea. Plaque-type morphea occurs in 36% to 48% of affected children.

Sclerodermiform dermatitis has no definitive cause; it is associated with certain drugs and toxic agents and possibly skin trauma. Some reports have suggested that 

 plays a role in the pathogenesis of morphea; however, treating morphea with the antibiotic therapy used for Lyme disease has not proved beneficial,

 and serological and polymerase chain reaction testing have failed to confirm the presence of 

 No immunological markers are clearly associated with morphea; however, elevated serum concentrations of transforming growth factor ?1 were found in patients with all subtypes of localized scleroderma irrespective of clinical disease activity.

 Plaque-like morphea usually begins with erythematous or purplish patches that evolve over weeks to months into firm, hyperpigmented or ivory plaques.

 The rash most commonly affects the trunk, neck, and extremities but may also involve the face. Superficial facial involvement is termed 

, whereas progressive atrophy of one side of the face is known as Parry-Romberg syndrome. Associations between facial scleroderma and neurological symptoms (including headache, trigeminal neuralgia, myopathy of external eye muscles, and seizure) have been reported.

Treatment of localized scleroderma is determined by the severity and duration of the lesions. Potent topical or injectable corticosteroids, while somewhat effective, are generally not recommended because they may contribute to local atrophy. Topical calcipotriene ointment has been shown to halt the progression of morphea and linear scleroderma when used early in the disease course, especially when used twice daily under occlusion.

 For more extensive and disabling lesions, systemic therapy with methotrexate, corticosteroids, oral calci-triol, D-penicillamine, cyclosporine, interferon, or UV light may be required. For lesions overlying a joint (which pose a risk of contractures), physiotherapy, warm baths, massage, and exercise may be helpful. Surgical reconstruction may be needed for deforming lesions, after the active disease phase has abated and the child's growth is complete.

This child was treated with calcipotriene cream but was lost to follow-up. 

Bono W, Dupin N. Localized scleroderma (morphea) [in French]. Presse Med.    2006;35(12, pt 2):1923-1928. 
    

Ruiz-Sandoval JL, Romero-Vargas S, Gutierrez-Aceves GA, et al. Linear scleroderma    en coup de sabre: neurological symptoms, images and review [in Spanish].    Rev Neurol. 2005;41:534-537. 
    

    Al-Khenaizan S, Al-Watban L. Parry-Romberg syndrome. Overlap with linear    morphea. Saudi Med J. 2005;26:317-319. 
    

Rosenwasser TA, Eizen AZ. Scleroderma. In: Fitzpatrick, Eizen AZ, Wolff K, eds.    Fitzpatrick's Dermatology in General Medicine. New York: McGraw-Hill; 2003:    2156-2159. 
    

Black CM, Stephens CO. Systemic sclerosis (scleroderma) and related    disorders. In: Maddison PJ, Isenberg DA, Woo P, et al, eds. Oxford Textbook of    Rheumatology. Oxford, UK: Oxford Medical Publications; 1993:771. 
    

Paller AS, Mancini AJ, eds. Hurwitz Clinical Pediatric Dermatology: A Textbook    of Skin Disorders in Childhood and Adolescence. 3rd ed. Philadelphia: Elsevier    Saunders; 2006:595-599. 
    

Weide B, Schittek B, Klyscz T, et al. Morphoea is neither associated with features    of Borrelia burgdorferi infection, nor is this agent detectable in lesional skin    by polymerase chain reaction. Br J Dermatol. 2000;143:780-785.

    Uziel Y, Feldman BM, Krafchik BR, et al. Increased serum levels of TGFbeta1    in children with localized scleroderma. Pediatr Rheumatol Online J. 2007;5:22.    

Articles

Scleroderma may present at any age and can be localized or systemic. Localized scleroderma affects the skin, subcutaneous fascia, and occasionally muscle and bone. Systemic scleroderma is characterized by chronic disease with both skin changes and visceral abnormalities. Rarely, localized scleroderma may progress to systemic disease; however, screening for this form is unnecessary in patients who have no systemic symptoms.1-5

Localized Scleroderma

For 3 weeks, a 3-year-old African American boy had a mildly pruritic rash on his buttocks, lower extremities, upper thighs, and soles. The patient was initially seen at an urgent care center, where he was given amoxicillin for suspected scarlet fever. A week later, he presented to the emergency department and was treated with griseofulvin for tinea corporis. A skin culture did not grow fungus.

The child's mother recalled that the rash started as a single patch on his lower back and that the child had an upper respiratory tract infection 4 weeks earlier.

The rash consisted of annular hyperpigmented plaques of 0.5 to 3 cm with dusky centers and whitish borders with scale. There was no mucosal membrane or scalp involvement. The rest of the examination findings were unremarkable. Pityriasis rosea was diagnosed.

Pityriasis rosea was first described by Gilbert in 1860 as pink scale. There may be several causative agents. A causal association between pityriasis rosea and active human herpesvirus (HHV) 7 and HHV6 has been suggested but not corroborated.

 Pityriasis rosea is more common in the fall and spring. Half of all cases occur in patients younger than 20 years; females are affected more than males. An upper respiratory tract infection precedes the illness in up to 68% of cases.

 The disease may pre- sent initially with mild prodromal symptoms, such as fatigue, headache, nausea, anorexia, chills, and arthralgia. Lymphadenopathy may occur before the onset of the rash.

Pityriasis rosea usually starts with a herald patch--which is typically the largest lesion and is commonly mistaken for tinea corporis. This is followed within 1 to 2 weeks by a papulosquamous plaque-like rash that lasts for about 6 to 12 weeks. In children, especially African American children,

 the lesions may be generalized or atypical in morphology and distribution, unlike the classic ovoid lesions in a Christmas tree pattern seen on the trunk of adults.

Treatment is unnecessary, but topical corticosteroids or oral antihistamines may be used for pruritus. Moisturizers may also be helpful. In one study, erythromycin used for 2 weeks resulted in complete resolution of the rash in 73% of patients, while the resolution rate was 0% in the placebo group.

 Azithromycin did not provide the same efficacy.

 Another study concluded that high-dose acyclo- vir may be effective if started within the first week of the rash when HHV replicative activity is very high; however, this study relied solely on observation and counting of lesions to determine resolution.

This patient was given diphenhydramine (5 mg/kg/d PO divided q6h) to relieve his itching. At follow-up 6 weeks later, the rash had completely resolved.	*

 Broccolo F, Drago F, Careddu AM, et al. Additional evidence that pityriasis rosea is associated with reactivation of human herpesvirus-6 and -7. 

 Sharma PK, Yadav TP, Gautam RK, et al. Erythromycin in pityriasis rosea: a double-blind, placebo-controlled clinical trial. 

 Amer A, Fischer H, Li X. The natural history of pityriasis rosea in black American children: how correct is the "classic" description? 

 Amer A, Fischer H. Azithromycin does not cure pityriasis rosea. 

 Drago F, Vecchio F, Rebora A. Use of high-dose acyclovir in pityriasis rosea. 

Pityriasis Rosea in a Young Boy

FigureFor 3 weeks, a 3-year-old African American boy had a mildly pruritic rash on his buttocks, lower extremities, upper thighs, and soles. The patient was initially seen at an urgent care center, where he was given amoxicillin for suspected scarlet fever. A week later, he presented to the emergency department and was treated with griseofulvin for tinea corporis. A skin culture did not grow fungus. 

 The child's mother recalled that the rash started as a single patch on his lower back and that the child had an upper respiratory tract infection 4 weeks earlier. 

The rash consisted of annular hyperpigmented plaques of 0.5 to 3 cm with dusky centers and whitish borders with scale. There was no mucosal membrane or scalp involvement. The rest of the examination findings were unremarkable. Pityriasis rosea was diagnosed. 

 Pityriasis rosea was first described by Gilbert in 1860 as pink scale. There may be several causative agents. A causal association between pityriasis rosea and active human herpesvirus (HHV) 7 and HHV6 has been suggested but not corroborated.

Pityriasis rosea is more common in the fall and spring. Half of all cases occur in patients younger than 20 years; females are affected more than males. An upper respiratory tract infection precedes the illness in up to 68% of cases.

The disease may present initially with mild prodromal symptoms, such as fatigue, headache, nausea, anorexia, chills, and arthralgia. Lymphadenopathy may occur before the onset of the rash. 

Pityriasis rosea usually starts with a herald patch- which is typically the largest lesion and is commonly mistaken for tinea corporis. This is followed within 1 to 2 weeks by a papulosquamous plaque-like rash that lasts for about 6 to 12 weeks. In children, especially African American children,

the lesions may be generalized or atypical in morphology and distribution, unlike the classic ovoid lesions in a Christmas tree pattern seen on the trunk of adults.

 Treatment is unnecessary, but topical corticosteroids or oral antihistamines may be used for pruritus. Moisturizers may also be helpful. In one study, erythromycin used for 2 weeks resulted in complete resolution of the rash in 73% of patients, while the resolution rate was 0% in the placebo group.

Azithromycin did not provide the same efficacy.

Another study concluded that high-dose acyclovir may be effective if started within the first week of the rash when HHV replicative activity is very high; however, this study relied solely on observation and counting of lesions to determine resolution.

This patient was given diphenhydramine (5 mg/kg/d PO divided q6h) to relieve his itching. At follow-up 6 weeks later, the rash had completely resolved. 

Broccolo F, Drago F, Careddu AM, et al. Additional evidence that pityriasis    rosea is associated with reactivation of human herpesvirus-6 and -7. J Invest    Dermatol. 2005;124:1234-1240. 
    

Sharma PK, Yadav TP, Gautam RK, et al. Erythromycin in pityriasis rosea:    a double-blind, placebo-controlled clinical trial. J Am Acad Dermatol. 2000;    42(2 pt 1):241-244. 
    

Amer A, Fischer H, Li X. The natural history of pityriasis rosea in black    American children: how correct is the "classic" description? Arch Pediatr Adolesc    Med. 2007;161:503-506. 
    

    Amer A, Fischer H. Azithromycin does not cure pityriasis rosea. Pediatrics.    2006;117:1702-1705.

    Drago F, Vecchio F, Rebora A. Use of high-dose acyclovir in pityriasis rosea.    J Am Acad Dermatol. 2006;54:82-85.    

Anne Sveen, MD

Articles

Localized Scleroderma

Pityriasis Rosea in a Young Boy

Pityriasis Rosea in a 3-Year-Old Boy