 |
|
|
|
|
|
|
|
|
 |
 |
 |
 |
 |
 |
 |
 |
|
Background
This amusing disease title stands for Acral Pseudolymphomatous Angiokeratoma of Children. It is a very rare and benign condition that predominately affects children but has recently been documented in adults.
OUTLINE
GROSS APPEARANCE/
CLINICAL VARIANTSCHARACTERIZATION GENERAL VARIANTS Acral angiokeratoma-like pseudolymphoma: One adolescent and two adults
Mikiko Okada, MD
Michitaka Funayama, MD, PhD
Muneo Tanita, MD
Kazuhiro Kudoh, MD, PhD
Setsuya Aiba, MD, PhD
Hachiro Tagami, MD, PhDSendai, Japan
J Am Acad Dermatol 2001;45:S209-11 Abstract quote
In 1988, Ramsay et al proposed an entity of acral pseudolymphomatous angiokeratoma of children (with an abbreviation of APACHE) for the unilateral multiple angiomatous papules affecting the acral region of the extremities of children.
We report here similar lesions that developed in the acral portions of 1 female adolescent and 2 women. Histopathologically, they showed pseudolymphomatous features rather than those of angiokeratoma.
Thus, the term should be acral angiokeratoma-like pseudolymphoma would be more appropriate than APACHE originally proposed.
LINEAR
- Linear acral pseudolymphomatous angiokeratoma of children (APACHE): further evidence that APACHE is a cutaneous pseudolymphoma.
Kiyohara T, Kumakiri M, Kawasaki T, Takeuchi A, Kuwahara H, Ueda T.
Department of Dermatology, the Division of Plastic Surgery, Fukui Medical University, Fukui, Japan.
J Am Acad Dermatol. 2003 Feb;48(2 Suppl):S15-7. Abstract quote
Acral pseudolymphomatous angiokeratoma of children (APACHE) is a recently recognized, rare clinical entity.
We report a case of APACHE in a 9-year-old Japanese girl with a dark red, linear plaque on the posterior area of the right lower leg.
Histopathologic findings revealed further evidence that APACHE was a cutaneous pseudolymphoma rather than a vascular neoplasm because of the presence of distinct primary and secondary lymphoid follicles, and thick-walled, long blood vessels lined with prominent plump endothelial cells similar to high endothelial venules in the paracortical area of the lymph node. In addition to previous descriptions, the patient presented with a distinct linear lesion. Vacuolar alteration of the basal cell layer and scattered eosinophilic bodies in the epidermis were observed, which suggested a lichenoid tissue reaction.
We believe that APACHE might show a distinct linear pattern accompanied by a lichenoid tissue reaction.
HISTOLOGICAL TYPES CHARACTERIZATION GENERAL Acral pseudolymphomatous angiokeratoma. A variant of the cutaneous pseudolymphomas.
Kaddu S, Cerroni L, Pilatti A, Soyer HP, Kerl H.
Department of Dermatology, University of Graz, Austria.
Am J Dermatopathol 1994 Apr;16(2):130-3 Abstract quote
APACHE (acral pseudolymphomatous angiokeratoma of children) is a recently described benign cutaneous condition. Only a few cases have been documented to date. The disease has been previously reported in children between 2 and 13 years of age as unilateral eruptions of multiple angiomatous papules on acral sites of the hands and feet.
We herein report two cases of APACHE, one occurring in a 16-year-old boy and the other in a 64-year-old woman. Unlike the previously reported cases, our female patient presented in adulthood with a single lesion on the back. Histopathologic and immunohistochemical investigations reveal characteristic features, allowing APACHE to be included among the group of cutaneous pseudolymphomas.
SPECIAL STAINS/
IMMUNOPEROXIDASE/
OTHERCHARACTERIZATION SPECIAL STAINS IMMUNOPEROXIDASE
- Acral pseudolymphomatous angiokeratoma of children: immunohistochemical and clonal analyses of the infiltrating cells.
Hagari Y, Hagari S, Kambe N, Kawaguchi T, Nakamoto S, Mihara M.
Department of Dermatology, Faculty of Medicine, Tottori University, Yonago, Japan.
J Cutan Pathol. 2002 May;29(5):313-8. Abstract quote
BACKGROUND: Acral pseudolymphomatous angiokeratoma of children (APACHE) is a disorder characterized clinically by red nodules and histopathologically by a massive subepidermal lymphohistiocytic infiltrate. Although it was initially thought to be a vascular nevus, it has never been regarded as a pseudolymphoma. Case report: We report a 7-year-old-girl with small red nodules on the dorsum of the right foot and a 73-year-old man with asymptomatic brown-red nodules on the lower extremities.
RESULTS: Histopathologic examination revealed a massive lymphohistiocytic infiltrate with plasma cells, some eosinophils, or a multinucleated giant cell immediately beneath the epidermis. Thick-walled vessels were observed in the infiltrate. These characteristics are identical to those of acral pseudolymphomatous angiokeratoma of children. The infiltrate was composed mainly of equal numbers of CD4+ or CD8+ T cells and equal numbers of B cells stained for kappa or lambda light chains. PCR amplification of rearranged immunoglobulin heavy chain genes or T-cell receptor gamma genes showed no evidence of clonality, suggesting that these infiltrates were polyclonal both for B and T cells.
CONCLUSIONS: Our data support the idea that this disorder represents a reactive process. The modified term 'papular angiolymphoid hyperplasia' would define this disorder more appropriately.Acral pseudolymphomatous angiokeratoma of children (APACHE): a case report and immunohistological study.
Hara M, Matsunaga J, Tagami H.
Department of Dermatology, Tohoku University School of Medicine, Sendai, Japan.
Br J Dermatol 1991 Apr;124(4):387-8 Abstract quote
Acral pseudolymphomatous angiokeratoma of children (APACHE) is a new clinical entity that is characterized by angiomatous papules on the extremities.
We report a case APACHE in a 14-year-old Japanese girl with asymptomatic red and violaceous papules and nodules on the ring finger of the left hand. Histological and immunohistochemical studies of our patient indicated that APACHE is a pseudolymphoma rather than an angiokeratoma.
Henry JB. Clinical Diagnosis and Management by Laboratory Methods. Twentieth Edition. WB Saunders. 2001.
Rosai J. Ackerman's Surgical Pathology. Ninth Edition. Mosby 2004.
Sternberg S. Diagnostic Surgical Pathology. Fourth Edition. Lipincott Williams and Wilkins 2004.
Robbins Pathologic Basis of Disease. Seventh Edition. WB Saunders 2005.
DeMay RM. The Art and Science of Cytopathology. Volume 1 and 2. ASCP Press. 1996.
Weedon D. Weedon's Skin Pathology Second Edition. Churchill Livingstone. 2002
Fitzpatrick's Dermatology in General Medicine. 5th Edition. McGraw-Hill. 1999.
Weiss SW and Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors. Fourth Edition. Mosby 2001.
Basic Principles of Disease
Learn the basic disease classifications of cancers, infections, and inflammation
Commonly Used Terms
This is a glossary of terms often found in a pathology report.Diagnostic Process
Learn how a pathologist makes a diagnosis using a microscopeSurgical Pathology Report
Examine an actual biopsy report to understand what each section meansSpecial Stains
Understand the tools the pathologist utilizes to aid in the diagnosisHow Accurate is My Report?
Pathologists actively oversee every area of the laboratory to ensure your report is accurate
Got Path?
Recent teaching cases and lectures presented in conferences
Pathologists Who Make A Difference
Search for a Physician Specialist
Last Updated June 9, 2005
Send mail to The Doctor's Doctor with questions or comments about this web site.
Read the Medical Disclaimer.
Copyright © The Doctor's Doctor