Case 6 -
T-cell Rich Angioma-like Polypoid Pseudolymphoma
Click on each slide thumbnail image for an enlarged view
Female age 36 with an erythematous nodule on the shoulder present for "some months". The clinical
assessment was possible pyogenic granuloma. The excised lesion was received in consultation with concern
for a lymphomatous process. The lesion has not recurred during more than 6 years of follow-up and no
additional lesions have been noted.
Case 6 - Slide 1
The sections reveal a polypoid lesion with a nodular dermal infiltrate composed of lymphocytes admixed
with small vessels. At most, minimal cytologic atypia was noted in the infiltrate. A focal area of
necrosis with surrounding fibrosis was also seen.
Immunohistochemical studies revealed that the lymphocytic infiltrate consists primarily CD3-positive T
cells with small nodules of CD20-positive B cells admixed. Both B- and T-cell gene re-arrangement
studies revealed a polyclonal population.
On the basis of the histologic and immunohistochemical images presented, the differential diagnosis
- Cutaneous lymphoma
- Acral pseudolymphomatous angiokeratoma of children (APACHE)
- Angiolymphoid hyperplasia with eosinophilia
- Benign cutaneous lymphoid hyperplasia
- Lobular capillary hemangioma / pyogenic granuloma
- T-cell rich angioma-like polypoid pseudolymphoma
T-cell Rich Angioma-like Polypoid Pseudolymphoma
We believe that this case may represent a novel lesion that we have provisionally termed T-cell rich
angioma-like polypoid pseudolymphoma. The most important point is that this lesion appears to behave in
a benign fashion. We have accumulated 13 strikingly similar cases which will be discussed further below.
The two primary cutaneous B-cell lymphomas that would be diagnostic considerations in this case would
be follicular center lymphoma and marginal zone B-cell lymphoma in the WHO-EORTC classification
. This case lacks the expanded germinal centers of follicular lymphoma and the expanded
marginal zone with lymphoplasmacytoid forms of marginal zone lymphoma. In addition, cytologic atypia is
minimal and the B-cells constitute only a minority of the cells by immunohistochemistry. This case is
not characteristic or particularly suggestive of any described form of T-cell lymphoma.
Acral pseudolymphomatous angiokeratoma of children (APACHE):
This lesion was originally described in 1988 as grouped angiomatous papules on acral sites with a
keratotic surface or collar around the base, suggestive of angiokeratoma of Mibelli.
has also been described under a variety of suggested monikers including "small papular pseudolymphoma
"acral angiokeratoma-like pseudolymphoma
" "acral pseudolymphomatous
angiokeratoma ," "papular angiolymphoid proliferation with epithelioid features in adults and
children (PALEFACE) ," and
"papular angiolymphoid hyperplasia ." Although APACHE
was considered a vascular neoplasm initially, it is now considered a pseudolymphoma rather than an
angiokeratoma. Immunohistochemical studies showed an admixture of B-and T-cells in varying proportions.
Although, the present case shares many features of this entity, APACHE is described as multiple papules
primarily in children (but may occur in adults on occasion), and is present at acral sites. The present
case occured in an adult and on the shoulder.
Lobular capillary hemangioma / pyogenic granuloma:
This was primarily a clinical concern. The histologic features did not show a well-developed lobular
capillary vascular proliferation characteristic of this entity .
Angiolymphoid hyperplasia with eosinophilia / epithelioid hemangioma (ALHE):
While there is a definite angiomatous/vascular component to this case, it lacks the characteristic
plump endothelial cells of ALHE which are epithelioid in appearance and often have prominent cytoplasmic
vacuoles. Instead, the vessels and endothelial cells of this case resemble the high endothelial venules
of the paracortical area of the lymph nodes. The stromal cellular infiltrate of ALHEalso has prominent
eosinophils and mast cells, which are absent in this case. Finally, ALHE is usually encountered in the
head and neck region rather than the shoulder. 
Benign cutaneous lymphoid hyperplasia (BCLH):
BCLH is a diagnostic consideration. However, the polypoid nature of the present case, lack of
germinal center formation and vascular pattern would be somewhat unusual for BCLH. A similar
proliferation of small blood vessels is observed in the entity angioplasmocellular hyperplasia,
 but the present case lacks endothelial cells with vacuolated cytoplasm and a predominance
of plasma cells. 
We (Eduardo Calonje and myself) have now collected a series of 13 lesions with strikingly similar
features to that presented above. While these lesions share features with APACHE, we believe them to be
distinct. Since the original description of APACHE, an additional 23 histologically similar cases of
this benign and non-progressive entity have been reported in the international literature, 18 of which
conformed to the classical presentation described above.
However, rare cases of
APACHE are described as solitary lesions (n=3),
occurring in adults (n=6)
and at non-acral sites (n=4).
The 3 described solitary cases
show striking clinical and histological resemblance to our collection of 13 cases, which we
believe are dissimilar in terms of clinical presentation, but likely related to APACHE given the
overlapping histologic features. This may imply that both entities belong to a wider spectrum of related
Our collected series of 13 cases includes 10 females and 3 males ranging in age from 16 to 71 (median,
41). Each presented as solitary polypoid erythematous papule ranging in size from 2.5 to 9 mm (mean, 7.5
mm). They occurred primarily from the trunk or head and neck regions. The majority were excised with a
clinical impression of pyogenic granuloma, but were sent in consultation with concern for a lymphomatous
process. All were treated by excision and none has recurred with median follow-up of 24 months
indicating a benign nature.
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