Spontaneous eruptive disseminated lobular capillary haemangioma

Abstract

Lobular capillary haemangioma (LCH), previously known as pyogenic granuloma, is a benign vascular tumour of the skin or mucosa. We report a patient with spontaneous eruption of LCH, a rare occurrence, which resolved probably due to reverse koebnerisation.

Background

Lobular capillary haemangioma (LCH) is a common benign vascular lesion.¹ Its previous term, pyogenic granuloma, for the same condition, is a misnomer, as the lesions are neither pyogenic nor granulomatous. Most lesions are solitary, usually arise at the site of trauma and occur in younger age groups. They are typically bright red, pedunculated papules or nodules and are surrounded by an epidermal collarette. They are partially blanchable and non-pulsatile. The common sites of occurrence are the extremities, face and upper trunk. Mucosal lesions tend to commonly occur in the oral cavity and the perianal mucosa. Oral lesions tend to grow rapidly, often ulcerate and can be mistaken for a malignancy.² Development of multiple lesions is more unusual and poses a diagnostic challenge, and management of the lesions becomes tedious. We report this uncommon subset of the disease with 176 lesions disseminated all over the body.

Case presentation

A female patient in her 30s presented to our department with a history of eruption of multiple red skin lesions over the back and face, which gradually progressed to involve the arms, forearms, trunk and lower limbs bilaterally within 2 months. She also had a history of bleeding from the lesions on touch, following which the lesions increased in size, became hyperpigmented and warty. The lesions were painless and not itchy. There were no systemic symptoms or any antecedent history of trauma, new drug intake, chronic medications, recent loss of weight or appetite, drug abuse or sexual promiscuity. The patient is married and her partner is asymptomatic. There is no history of similar symptoms in the past. The patient does not have any other comorbidities.

On clinical examination, multiple lesions were present (totalling 176); they were bright red, nodular lesions, both pedunculated and sessile over the face, scalp, back (figure 1A–C), chest (figure 2), abdomen (figure 3) and bilateral extremities. Few nodules had a lobulated surface. They were non-tender, non-pulsatile and partially blanchable. A few hyperpigmented verrucous nodules were noted over the face and upper back.

Figure 1.

(A) Multiple lobular capillary haemangiomas (LCHs) seen over the patient’s neck and upper back before radiofrequency removal. (B) Multiple LCHs seen over the patient’s mid-back. (C) Multiple LCHs seen over the patient’s lower back.

Figure 2.

Multiple lobular capillary haemangiomas seen over the patient’s upper chest.

Figure 3.

Multiple lobular capillary haemangiomas seen over the patient’s abdomen.

Investigations

Hemogram, chest radiograph, ultrasound of the abdomen, liver function test, renal function test, fasting and post-prandial blood sugar tests were all within normal limits. Serological tests for HIV, hepatitis B and hepatitis C and rapid plasma reagin tests were found to be non-reactive. Blood and urine cultures showed no growth. Biopsy of the lesion showed normal epidermis and lobules of variably dilated blood-filled capillaries in the oedematous superficial and mid-dermis. Prominent endothelial cells with occasional mitosis and no atypia and scattered spindle cells were seen. Few neutrophils and lymphocytes were also appreciated. The Warthin-Starry and Giemsa stains were negative for bacillary angiomatosis.

Differential diagnosis

Differential diagnoses of multiple LCHs, bacillary angiomatosis (BA) and Kaposi sarcoma (KS) were made with the above-mentioned history and clinical findings.

KS is a multifocal, endothelial proliferation caused by human herpes virus-8, mostly with cutaneous involvement with or without visceral extension. It is associated with HIV coinfection and presents as multifocal dark blue or purple patches fusing to form plaques, then nodules, seen commonly in the extremities. They can ulcerate or fungate leaving pigmented scars. Lymph nodes, mucosae and viscera may be involved. Local or generalised lymphadenopathy is common and histopathological examination (HPE) shows extensive vessel expansion, lined by single-layered plump endothelial cells, chronic lymphoplasmacytic infiltrate with extravasated red blood cell and haemosiderin deposits. HPE also shows mitotic activity, periodic acid-Schiff positivity, diastase resistance and amorphous eosinophilic globules. Immunohistochemistry shows positivity for CD31, CD34, factor VIII-related antigen and podoplanin.³

BA is a rare disease, often seen in patients with AIDS and other immunocompromised patients. The disease is characterised by the development of friable angiomatous papules and nodules. It is caused by the Bartonella spp. Following septicaemia, it presents as small papules or dermal nodules, singly or in crops, which can involve any site including the mucosa. Local lymphadenopathy is often seen and the HPE shows lobular proliferation of small blood vessels with swollen endothelial cells. Pale epithelioid endothelial cells, neutrophils and nuclear dust are seen throughout the lesions with violaceous amorphous aggregates of bacilli which are positive with either Giemsa or Warthin-Starry stains.⁴

With negative Warthin-Starry and Giemsa stains on the biopsy specimen and as the patient was non-reactive for HIV test, we were able to rule out the possibility of BA and KS.

Treatment

Large, pedunculated facial lesions, five in number and two upper back lesions of cosmetic concern were treated with radiofrequency (RF) ablation.

Outcome and follow-up

The patient was offered oral propranolol therapy, for which she refused. After 1 month, her lesions completely regressed without any further intervention (figure 4A–D).

Figure 4.

(A) Image showing complete resolution of lesions on the upper back. (B) Image showing complete resolution of lesions on the upper chest. (C) Image showing complete resolution of lesions on the abdomen. (D) Image showing complete resolution of lesions on the back.

Discussion

LCH usually pursues a benign course, although bleeding and ulceration can occur. Eruptive disseminated LCH is characterised by multiple widespread lesions, sudden in onset. They are more common in the younger age groups and children. Rare associations have been reported such as following multiple traumatic injuries, following etretinate and isotretinoin therapy, and internal malignancies.⁵ There have been reports of LCH arising within port-wine stains, following the treatment of port-wine stains with carbon dioxide laser and exfoliative dermatitis, after a hypersensitivity drug reaction and after removal of melanocytic nevus.^{2 5–7}

Our case was peculiar for its spontaneous onset of lesions without any associated trauma, skin or systemic disease and the resolution of lesions within 4 weeks of RF ablation done only on a few lesions. The resolution of lesions after RF ablation of only a few large lesions raises the role of reverse Koebner’s phenomenon as a possible mechanism behind the spontaneous resolution of the lesions. This is seen in other conditions like granuloma annulare, vitiligo, psoriasis and oral lichen planus.^8–11

Although a few cases have been reported with innumerable LCHs without any predisposition, they have been treated with cryotherapy, pulsed dye laser and propranolol.^{2 6} In these reported cases, it has not been mentioned if the lesions were treated simultaneously or sequentially or if any lesions resolved spontaneously. Hence, we are of the opinion that our case is the first reported for possible reverse koebnerisation or coincidental spontaneous resolution of the lesions.

The pathogenesis of multiple and satellite LCHs is not understood very well. Various theories have been proposed like trauma; hormonal influences; certain medications like panitumumab, 5-fluorouracil, mitoxantrone, docetaxel, paclitaxel, mammalian target of rapamycin inhibitors, vemurafenib, blinatumomab and ramucirumab; certain viruses; underlying microscopic arteriovenous malformations; the production of angiogenic growth factors and cytogenetic abnormalities. The overexpression of transcription factors P-ATF2 and STAT3 may also play a role in tumourigenesis.¹² One such theory is the release of various endogenous substances including angiogenic factors following trauma which can cause disturbances in the vascular system of the affected area.¹³ Certain neoplastic diseases like multiple myeloma, Hodgkin’s disease, chronic lymphocytic leukaemia, disseminated melanoma and inflammatory disorders such as psoriasis vulgaris, atopic dermatitis and rosacea may release angiogenic factors leading to the development of vascular lesions like LCH.^{14 15} Hence, an earnest effort should be made to identify such underlying disorders appropriately.

Learning points.

• In cases with multiple lobular capillary haemangiomas, it is important to wait and watch rather than start aggressive treatment of multiple lesions, which is tedious, as the lesions can resolve spontaneously.

• Biopsy and blood investigations are a must to rule out other possible differentials and to rule out any underlying malignancies, especially in the older age groups.

• One of the mechanisms of spontaneous resolution could be due to reverse koebnerisation at a distant site.

Ethics statements

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