Angiokeratoma-like purpuric palmar nodules following chemotherapy.

We describe a patient with leukemia undergoing chemotherapy who developed painful purpuric nodules of the digits. These findings were concerning for endocarditis (clinically) and angiokeratomas on gross histology. After extensive evaluation, we report the development of painful purpuric nodules as a likely side effect of the patient's therapeutic regimen (hydroxyurea, danorubicin, cytarabine, and methotrexate).

[Solitary angiokeratoma of the tongue]. / Solitair angiokeratoom van de tong.

A 41-year-old woman, who was referred with a reddish purple like lesion on the left side of the tongue, appeared to have an angiokeratoma after histopathological examination. Because of the benign character of this lesion and the absence of any complaints, no adjuvant treatment after excisional biopsy was indicated. Angiokeratomas rarely appear as solitary oral lesions. More often they are seen as part of an underlying systemic disease, mostly Fabry disease. Due to widespread skin involvement of angiokeratomas with Fabry disease, referral to a dermatologist is indicated when oral lesions are encountered. Esthetically unpleasing or painful angiokeratomas can be locally excised or treated by laser- or cryotherapy.

Genital Rejuvenation of Scrotal and Vulvar Angiokeratomas: Laser Therapy of Scrotum and Vulva Lesions.

Genital rejuvenation requires medical and procedural treatments that can improve the morphology or aesthetics of the scrotum and vagina. Angiokeratomas are benign vascular lesions that my occur on the penis, scrotum, and vulva. These lesions can be of either cosmetic concern or medical significance if they bleed. Laser therapy can be successfully utilized for the treatment of genital angiokeratomas in men and women.

Angiokeratoma Circumscriptum Naeviforme Presenting as a Dark Warty Plaque on the Leg.

Dear Editor, A 45-year-old man presented with a large, dark, keratotic, warty, and friable plaque on the distal posterior aspect of the left leg (Figure 1, a). The patient reported that the lesion was not present at birth but had appeared approximately at the age of three as an erythematous patch that progressively grew over the time. During adolescence, the surface of the lesion became rough and warty and was easily traumatized due to its location, resulting in recurrent bleeding episodes over a period of years. For this reason, the patient requested lesion removal. The patient did not report any other significant comorbidity, and physical examination revealed no other abnormalities. A shaving biopsy of the lesion was performed, and histopathology highlighted ectatic vascular spaces with some luminal red blood cell beneath a papillomatous and hyperkeratotic epidermis (Figure 1, b). Based on clinical and histopathological features, a diagnosis of angiokeratoma circumscriptum naeviforme (ACN) was established. ACN is one of the five disorders belonging to the group of angiokeratomas (AKs) that also include AK of Mibelli, AK of Fordyce, solitary or multiple AK, and AK corporis diffusum. Among these variants, ACN is the rarest and is seldom studied (1). AKs are benign vascular anomalies of the superficial vascular plexus that appear as dark red papules and plaques arranged either discretely or in clusters. ACN lesions are typically situated unilaterally on the lower limbs, especially on the legs and feet, but can occasionally occur elsewhere. Lesions are generally noted in early childhood. The early lesions are flat and reddish in color, while older lesions become increasingly studded and acquire a verrucous or warty surface. There is no tendency of spontaneous improvement, and minor traumas can easily cause beading and infection (2). While the plaques were linear in disposition in most of the cases reported in the literature, a peculiar feature of our case was the isolated, round, and giant appearance of the plaque. Even though ACN is not typically associated with other abnormalities, coexistence with other vascular malformations has been reported in some cases, including AK of Fordyce, Cobb syndrome, Klippel-Trenaunay syndrome, nevus flammeus, infantile hemangioma, and traumatic arteriovenous fistula (3). From a histological point of view, ACN appears as dilated dermal papillary capillaries drained by dilated venules. The overlying epidermis shows a variable degree of acanthosis, papillomatosis, and compact hyperkeratosis. Typically, the deep dermis and hypodermis are not involved, helping distinguish them from verrucous hemangioma (4). Lichen simplex chronicus, verrucous carcinoma, and verrucous melanoma must also be considered in the differential diagnosis (5). Recurrent bleeding or cosmetic reasons are common indications for treatment. Surgical excision represents the most effective option. Other possibilities include diathermy, electrocautery, cryosurgery, or laser (6). In our case, there was no macroscopic residual disease after the shaving biopsy. A collagen dressing was applied, and the wound underwent second intention healing in three weeks. There was no evidence of local recurrence after 18 months. References: Das A, Mondal AK, Saha A, Chowdhury SN, Gharami RC. Angiokeratoma circumscriptum neviforme: An entity, few and far between. Indian Dermatol Online J. 2014;5:472-4. Mittal R, Aggarwal A, Srivastava G. Angiokeratoma circumscriptum: a case report and review of the literature. Int J Dermatol. 2005;44:1031-4. Wankhade V, Singh R, Sadhwani V, Kodate P, Disawal A. Angiokeratoma circumscriptum naeviforme with soft tissue hypertrophy and deep venous malformation: A variant of Klippel-Trenaunay syndrome? Indian Dermatol Online J. 2014;5(Suppl 2):S109-S112. Oppermann K, Boff AL, Bonamigo RR. Verrucous hemangioma and histopathological differential diagnosis with angiokeratoma circumscriptum neviforme. An Bras Dermatol. 2018;93:712-5. Goldman L, Gibson SH, Richfield DF. Thrombotic angiokeratoma circumscriptum simulating melanoma. Arch Dermatol. 1981;117:138-9. del Pozo J, Fonseca E. Angiokeratoma circumscriptum naeviforme: successful treatment with carbon-dioxide laser vaporization. Dermatol Surg. 2005;31:232-6.

Angiokeratoma circumscriptum.

Angiokeratoma circumscriptum is the rarest variant of angiokeratoma. It usually affects females and it is characterized by dark-red to blue-black confluent papules or nodules on lower limbs in a segmental and unilateral distribution. We describe the clinical and histopathological findings in a patient with angiokeratoma circumscriptum and discuss the etiology, associations, diagnosis, differential diagnosis, and treatment.

Angiokeratomas and treatment with enzyme replacement therapy in a patient with Fabry disease.

Angiokeratomas are the cutaneous hallmark of Fabry disease. Although it is well established that enzyme replacement therapy (ERT) prevents or slows the progression of disease on target organs in the majority of patients, the long-term effect of ERT on angiokeratomas remains unknown. We present a patient diagnosed with Fabry disease at age 11, with rapid progression of new angiokeratomas in typical regions before beginning treatment with ERT. To date, our patient has been treated with ERT for 10 years. During the treatment period, new angiokeratomas have not arisen nor have existing ones enlarged during puberty, adolescence, and early adulthood. Furthermore, partial regression of the angiokeratomas has occurred in association with regression of left ventricular hypertrophy and anhidrosis. Overall, this case suggests that long-term ERT could stop the progression of angiokeratomas and induce their partial regression but does not produce complete resolution. Importantly, regression of angiokeratomas might be a marker of systemic target-organ efficacy of ERT.

Use of topical rapamycin in acral pseudolymphomatous angiokeratoma of children (APACHE): A report of two cases and review of the literature.

Acral pseudolymphomatous angiokeratoma of children (APACHE) is a rare, benign disease characterized clinically by multiple, asymptomatic, erythematous papules in the acral regions. We report APACHE in a 12-year-old girl with erythematous-violaceous papules on the lateral dorsum of her foot and toes, and a 3-year-old girl with erythematous papules on the plantar aspect of her foot. Topical rapamycin ointment improved the lesions and both patients tolerated the medication well. Topical rapamycin appears to be a potentially efficacious, well-tolerated, non-invasive therapy in APACHE, although further studies are needed.