Rumple Leede

J.A. Schneider, DO Medical Director, Adolescent Medicine Mobile Health Outreach

St. Vincent Hospital - Jacksonville, FL CAPT, MC, USN (Ret)

Asst. Professor, Division of Adolescent Medicine, University of Florida (Ret)

Rumple Leede l Rumple is a seven- year-old male who

presented to his pediatrician with a rash of 3 months duration.

l This child was entirely asymptomatic and has been in excellent health his entire life.

l There had been no recent illnesses, trauma, nor reported exposures to his skin.

l His past medical history, family history, and review of systems was non-contributory.

Rumple Leede (continued)

l The present history indicated that this ‘rash” began as darkened areas on his right leg that slowly enlarged over a period of 2-3 months.

l Failure of the rash to resolve prompted the child’s mother to bring him to his pediatrician.

l The physical examination was entirely normal with the exception of the following: l Symmetrical reddish-brown macular lesion

over his distal right fibula and ankle l Non-palpable petecchiae on the periphery

of the macular lesions.

Rumple Leede (continued)

l Laboratory tests performed included a complete blood count, platelet count, bleeding time, prothrombin/ partial thromboplastin time, and urinalysis. All tests were normal.

Rumple Leede (continued)

Note the macular reddish-brown “bruise” over the distal fibula and dorsum of the right foot. The lesion is non-palpable

Close-up of “bruise.” Note the pin head size petecchiael lesions on the periphery

The thought process... l This sure doesn’t fit the picture for a

malignancy l The lesions are not palpable… doesn’t fit

the picture for Henoch Schonlein’s Purpura (HSP)

l No contacts… not likely phytophodermatitis l No medications… not fixed drug eruption l Persistent “bruising”…hmm...

The answer for this case is...

• Classified as one of the pigmented purpuras - a group of conditions with similar clinical and histological appearances

• Each condition is considered to be a capillaritis - superficial blood vessel inflammation with leakage of blood into the dermis

• Hemosiderin-laden macrophages are present on histology

Schamberg’s Disease Progressive pigmented purpura

• Is a benign, chronic condition characterized by petecchiae and hyperpigmented macules

• May occur at any age • Occurs 5 times more commonly in males.

The primary lesion are red-brown punctate lesions (“cayenne-pepper spots”) at the periphery of a red-brown hyperpigmented patch.

Schamberg’s Disease Progressive pigmented purpura

• Usually asymptomatic… occasionally pruritic

• Tends to be a persistent process with frequent flare-ups

Schamberg’s Disease Progressive pigmented purpura

The Pigmented Purpuras • Schamberg’s Disease • Pigmented Purpuric Lichenoid Disorder

(PPLD) of Gougerot and Blum • Purpura Annularis Telangiectodes

(Majocchi’s disease) • Lichen aureus • Eczematid-like purpura of Doucas and

Kapetankis (itching purpura)

References: Schamberg’s Disease

• Nagata K, Danno K, Tanaka S Unilateral Schamberg disease in a 14-year-old Japanese boy. J Dermatol, Jun 1999, 26(6) p348-51

• Zvulunov A, Avinoach I, Hatskelzon L, et al. Pigmented purpuric dermatosis (Schamberg's purpura) in an infant. Dermatol Online J, May 1999, 5(1) p2

• Bethea L Schamberg's disease: a case report. J S C Med Assoc, Nov 1995, 91(11) p469-70

• Aiba S, Tagami H Immunohistologic studies in Schamberg's disease. Evidence for cellular immune reaction in lesional skin. Arch Dermatol, Jul 1988, 124(7) p1058-62

• Newton RC, Raimer SS Pigmented purpuric eruptions. Dermatol Clin, Jan 1985, 3(1) p165-9

• Abeck D, Gross GE, Kuwert C, et al. Acetaminophen-induced progressive pigmentary purpura (Schamberg's disease). J Am Acad Dermatol, Jul 1992, 27(1) p123-4

References: Schamberg’s Disease

• Firouzi P, et al Pigmented purpuric eruptions of childhood: a series of cases and a review of the literature Pediatric Dermatology, Aug 2001 18 (4) p 299-304

• Eulalia Baselga, MD, Beth A. Drolet, MD, and Nancy B. Esterly, MD Purpura in infants and children J Am Acad Dermatol 1997;37:673-705

References: Schamberg’s Disease