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 and Clinical Variants
Histopathological Features and Variants
Special Stains/Immunohistochemistry/Electron Microscopy
Commonly Used Terms

PATHOGENESIS	CHARACTERIZATION
CLONAL ANALYSIS
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; Kambe Dermatologic Clinic, Tottori; and Departments of Dermatology and Pathology, Tottori Prefectural Central Hospital, Tottori, 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.

 

GROSS APPEARANCE/ CLINICAL VARIANTS	CHARACTERIZATION
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, PhD Sendai, 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.

 

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/ OTHER	CHARACTERIZATION
SPECIAL STAINS
IMMUNOPEROXIDASE
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.

Weedon D. Weedon's Skin Pathology. Churchill Livingstone. 1997.
Fitzpatrick's Dermatology in General Medicine. 5th Edition. McGraw-Hill. 1999.

Pseudolymphoma

Last Updated 8/6/2002

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