3
Journal of the American Academy of Dermatology Volume 29, Number 5, Part 2 44. Leaker B, McGregor A, Griffiths M, et al. Insidious loss of renal function in patients with anticardiolipin antibodies and absence of overt nephritis. Br J Rheumatol 1991; 30:422-5. 45. Hazeltine M, Rauch J, Danoff D, et at. Antiphospholipid antibodies in systemic lupus erythematosus: evidence of as- Amster et al. sociation with positive Coombs' and hypocomplementemia. J RheumatoI1988;15:80-6. 46. Asherson R, Hughes GRV. Hypoadrenalism, Addisons disease and antiphospholipid antibodies. J Rheumatol 1991;18:1-3. Eruptive pseudoangiomatosis: A unique childhood exanthem? Neil S. Prose, MD,a, b Whitney Tope, MD,c Sara E. Miller, PhD,d, e and Hideko Kamino, MDa, e Durham, North Carolina, and Portland, Oregon We describe three children with an acute onset and spontaneous resolution of angioma-like papules during an apparent viral illness. A biopsy specimen from one patient revealed a unique histologic appearance that consisted of dilated dermal blood vessels with plump hobnail- shaped endothelial cells. On the basis of the natural history and histopathologic features of this exanthem, we suggest the name eruptive pseudoangiomatosis. (J AM ACAD DERMATOL 1993;29:857-9.) In 1969, Cherry et aU described four children with an acute echovirus infection who had "heman- gioma-like" skin lesions. The lesions resolved spon- taneously within several days. We have recently ob- served three children with an identical eruption. CASE REPORTS Case 1 A 6-month-old white boy had two 4 mm bright-red papules on the left cheek that developed in 24 hours (Fig. 1). The lesions blanched completely with pressure and rapidly refilled from the center on release. The onset of the lesions coincided with the resolution of an upper respiratory infection. The lesions resolved completely in 3 days. Case 2 A6 1 h-month-old white boy with an upper respiratory infection had bright red papules on the trunk and From the Departments of Medicine (Dermatology),- Pediatrics,b Mi- crobiology,d and Pathology," Duke University Medical Center, and the Department of Dermatology," Oregon Health Sciences Univer- sity. Reprint requests: Neil S. Prose, MD, Box 3252, Duke University Med- ical Center, Durham, NC 27710. Copyright © 1993 by the American Academy of Dermatology, Inc. 0190-9622/93 $1.00 +.10 16/4/48323 extremities that developed during the course of 4 days. During the next 3 days several lesions resolved spontane- ously and several new lesions developed. Examination re- vealed six I to 2 mm, bright-red angiomatous papules on the hands, arms, and forehead (Fig. 2). The lesions blanched completely with pressure. One week later all le- sions had resolved. Case 3 A 6-year-old white boy had fever, temporal headache, severe vomiting, and malaise for 2 days. Examination re- vealed enlarged and erythematous tonsils. In addition, he had 10 blanchable angiomatous macules and papules on the face, shoulder, arm, and back. Skin biopsy specimens for light and electron micros- copy were obtained from lesions on the arm. Histologic examination revealed several dilated capillaries and small blood vessels in the upper reticular dermis but mainly in the papillary dermis (Fig. 3). The vessels were not increased in number. The vessels were lined by plump en- dothelial cells, some of which had a hobnail appearance (Fig. 4). The nuclei were slightly enlarged and a few en- dothelial cells were in mitosis. There was a sparse perivascular lymphohistiocytic infiltrate. The epidermis showed no pathologic changes. Electron microscopy revealed swollen endothelial cells with curly intermediate filaments that were 8 to 12 nm in diameter. These cytoplasmic filaments were believed to result from increased activity within the endothelial cells 857

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Page 1: Eruptive pseudoangiomatosis: A unique childhood exanthem?

Journal of the American Academy of DermatologyVolume 29, Number 5, Part 2

44. Leaker B, McGregor A, Griffiths M, et al. Insidious loss ofrenal function in patients with anticardiolipin antibodiesand absence of overt nephritis. Br J Rheumatol 1991;30:422-5.

45. Hazeltine M, Rauch J, Danoff D, et at. Antiphospholipidantibodies in systemic lupus erythematosus: evidence of as-

Amster et al.

sociation with positive Coombs' and hypocomplementemia.J RheumatoI1988;15:80-6.

46. Asherson R, Hughes GRV. Hypoadrenalism, Addisonsdisease and antiphospholipid antibodies. J Rheumatol1991;18:1-3.

Eruptive pseudoangiomatosis: A unique childhoodexanthem?Neil S. Prose, MD,a, b Whitney Tope, MD,c Sara E. Miller, PhD,d, e andHideko Kamino, MDa, e Durham, North Carolina, and Portland, Oregon

We describe three children with an acute onset and spontaneous resolution of angioma-likepapules during an apparent viral illness. A biopsy specimen from one patient revealed a uniquehistologic appearance that consisted of dilated dermal blood vessels with plump hobnail­shaped endothelial cells. On the basis of the natural history and histopathologic features ofthis exanthem, we suggest the name eruptive pseudoangiomatosis. (J AM ACAD DERMATOL1993;29:857-9.)

In 1969, Cherry et aU described four childrenwith an acute echovirus infection who had "heman­gioma-like" skin lesions. The lesions resolved spon­taneously within several days. We have recently ob­served three children with an identical eruption.

CASE REPORTS

Case 1

A 6-month-old white boy had two 4 mm bright-redpapules on the left cheek that developed in 24 hours(Fig. 1). The lesions blanched completely with pressureand rapidly refilled from the center on release. The onsetof the lesions coincided with the resolution of an upperrespiratory infection. The lesions resolved completely in3 days.

Case 2

A 61h-month-old white boy with an upper respiratoryinfection had bright red papules on the trunk and

From the Departments of Medicine (Dermatology),- Pediatrics,b Mi­crobiology,d and Pathology," Duke University Medical Center, andthe Department of Dermatology," Oregon Health Sciences Univer­sity.

Reprint requests: Neil S. Prose, MD, Box 3252, Duke University Med­ical Center, Durham, NC 27710.

Copyright © 1993 by the American Academy of Dermatology, Inc.0190-9622/93 $1.00 +.10 16/4/48323

extremities that developed during the course of 4 days.During the next 3 days several lesions resolved spontane­ously and several new lesions developed. Examination re­vealed six I to 2 mm, bright-red angiomatous papules onthe hands, arms, and forehead (Fig. 2). The lesionsblanched completely with pressure. One week later all le­sions had resolved.

Case 3

A 6-year-old white boy had fever, temporal headache,severe vomiting, and malaise for 2 days. Examination re­vealed enlarged and erythematous tonsils. In addition, hehad 10 blanchable angiomatous macules and papules onthe face, shoulder, arm, and back.

Skin biopsy specimens for light and electron micros­copy were obtained from lesions on the arm. Histologicexamination revealed several dilated capillaries and smallblood vessels in the upper reticular dermis but mainly inthe papillary dermis (Fig. 3). The vessels were notincreased in number. The vessels were lined by plump en­dothelial cells, some of which had a hobnail appearance(Fig. 4). The nuclei were slightly enlarged and a few en­dothelial cells were in mitosis. There was a sparseperivascular lymphohistiocytic infiltrate. The epidermisshowed no pathologic changes.

Electron microscopy revealed swollen endothelial cellswith curly intermediate filaments that were 8 to 12 nm indiameter. These cytoplasmic filaments were believed toresult from increased activity within the endothelial cells

857

Page 2: Eruptive pseudoangiomatosis: A unique childhood exanthem?

858 Prose et al.Journal of the American Academy of Dermatology

November 1993

Fig. 1. Case 1. Two blanchable angiomatous papules of I-day duration.Fig. 2. Case 2. Small blanchable angiomatous papules.Fig. 3. Case 3. Dilated blood vessels in papillary dermis and upper reticular dermis andsparse perivascular infiltrate.Fig. 4. Case 3. Higher magnification shows prominent endothelial cells lining the lumen ofa very dilated blood vessel. Some cells have a hobnail appearance.

and were not believed to be viral particles.2 No enterovi­rus particles were seen.

DISCUSSION

Acute hemangioma-like lesions were observed byCherry et aLl in four infants between the ages of 8and 11 months. They were described as 2 to 4 mmsparse erythematous papules surrounded by a 1 to 4mm halo ofblanching. Pressure on the center ofeachlesion resulted in complete blanching. Echovirus 25was isolated from two children, and echovirus 32 wasisolated from the other two children.

Cherryet a1. I performed no skin biopsies but rec­ognized the unique vascular appearance of the skinlesions and suggested that viral localization in endo­thelial cells caused capillary dilation. They hypo­thesized that the lesions resulted from either a direct

effect of viral infection on endothelial cells or anti­gen-antibody complexes binding to endothelium.

The appearance of the skin lesions in our patientsis remarkably similar to the appearance of thelesions reported by Cherry et al. l The clinical picturesuggested a viral illness in all three children, but ourattempt to document this in one patient was unsuc­cessful.

The histologic appearance of dilated blood vesselswith plump endothelial cells and no evidence of vas­cular proliferation is unique. Because the lesionshave the clinical appearance of angiomas but showno histologic evidence of an increased number ofblood vessels, we propose the name eruptivepseudoangiomatosis.

The histologic differential diagnosis of eruptivepseudoangiomatosis includes telangiectases and spi-

Page 3: Eruptive pseudoangiomatosis: A unique childhood exanthem?

Journal of the American Academy of DermatologyVolume 29, Number 5, Part 2

der angiomas. The lesions of eruptive pseudoangi­omatosis can be differentiated from telangiectasesby the presence ofendothelial cells that protrude intothe vessel lumen. In spider angiomas there are cen­trally located dilated arterioles with thin branches,without prominence of the endothelial lining.

Clinically, eruptive pseudoangiomatosis must bedifferentiated from bacillary angiomatosis, bartonel­losis, and pyogenic granuloma. In all three of thesedisorders the skin lesions are generally larger andlast considerably longer than the 7- to IO-day dura­tion that we observed in our patients. Spontaneousresolution does not occur. The occurrence of multi­ple pyogenic granulomas is relatively rare.3

Histologically, eruptive pseudoangiomatosisshares with bacillary angiomatosis and bartonellosisthe presence of a prominent endothelial lining.4

However, eruptive pseudoangiomatosis shows noblood vessel proliferation, inflammatory infiltratewith neutrophils, or granular deposits that representclustered bacilli. Pyogenic granuloma is character­ized by a dome-shaped proliferation of small blood

Prose et al.

vessels lined by oval endothelial cells. An epidermalcollarette and a variable degree of mixed inflamma­tory infiltrate may be present.

Although the data of Cherryet aLI implicated anenterovirus, the evidence linking the cutaneous le­sions to a systemic viral infection in our patients wascircumstantiaL It appears most likely that eruptivepseudoangiomatosis is a viral exanthem. However, itmay be an unusual cutaneous reaction pattern toseveral different organisms.

REFERENCES1. Cherry JD, Bobinski JE, Horvath FL, et al. Acute heman­

gioma-like lesions associated with ECHO viral infections.Pediatrics 1969;44:498-502.

2. Ghadially FN. Ultrastructural pathology of the cell. Stone­ham, Mass.: Butterworth-Heinemann, 1975:424-8.

3. Dillman AM, Miller RC, Hansen RC. Multiple pyogenicgranulomata in childhood. Pediatr DermatoI1991;8:28-31.

4. LeBoit PE, Berger TG, Egbert BM, et al. Bacillary angio­matosis: the histopathology and differential diagnosis of apseudoneoplastic infection in patients with human immuno­deficiency virus disease. Am J Surg Pathol 1989;13:909­20.

Granuloma annulare perforans in herpes zosterscarsDieter Krahl, MD, Wolfgang Hartschuh, MD, and Wolfgang Tilgen, MDHeidelberg, Germany

Granuloma annulare perforans limited to a thoracic dermatome that was previously involvedby herpes zoster occurred in a 51-year-old woman who also had Lennert's lymphoma. Of thevarious local granulomatous infiltrates described after herpes zoster, granuloma annulareperforans is unique, although ordinary granuloma annulare has been described in a few pa­tients. Ahigh incidence of specific and nonspecific reaction patterns in herpes zoster scars hasbeen described in patients with malignant lymphoma. In contrast to previous patients, all ofwhom had chronic lymphatic leukemia, our patient had Lennert's lymphoma. (J AM ACADDERMATOL 1993;29:859-62.)

Several variants ofgranuloma annulare (GA) canbe distinguished clinically and histopathologically,including the lichenoid, infiltrated, plaquelike, tu-

From the Universitals-Hautklinik, Ruprecht-Karls-University.Reprint requests: Dicter Krahl, MD, Universitats-Haulklinik, Vossslr.

2, D-6911S Heidelberg, Germany.Copyright ® 1993 by the American Academy of Dermatology, Inc.0190.9622/93 $1.00 +.10 16/4/48998

berous, subcutaneous, and perforating types withlimited, disseminated, or generalized distribution. 1

The chief histologic patterns are the palisadinggranuloma and the interstitial form. A rare epithe­lioid nodule type also occurs. Combinations of thesepatterns frequently occur within individual lesionsand in the same patient. Perforating granuloma an­nulare (PGA) shows transepidermal elimination ofdegenerated collagen from the center of palisadinggranuloma type lesions.

859