Nuchal Papules

CASE DESCRIPTION

Nuchal papules are a common clinical presentation in the dermatology outpatient department, with acne keloidalis nuchae (AKN) being the most common culprit. However, other entities such as folliculitis decalvans, hidradenitis suppurativa, dissecting cellulitis, acne conglobata, angiolymphoid hyperplasia with eosinophilia (ALHE), pyogenic granuloma, follicular cutaneous T-cell lymphoma and bacterial folliculitis may mimic AKN and confound the clinician. Herein, we present a clinicopathological challenge where the patient was initially diagnosed as AKN; however, dermoscopic and histopathological examination led to a change in the diagnosis.

A 26-year-old male reported with 6-month history of multiple painless papules and nodules over the nape of neck. The lesions gradually increased in size and number over the course of 6 months. The patient gave a history of keeping a short crop and wearing a collared shirt as a part of his job requirement, suggesting recurrent friction and trauma as an inciting factor. He denied any history of hair loss or similar lesions over his face, back, axilla or inguinal region. Physical examination revealed multiple, polysized, firm, non-tender, skin coloured to erythematous papules and nodules over the nape of neck [Figure 1]. There was no lymphadenopathy. Based on history and clinical examination, the patient was diagnosed with a case of AKN and empirically managed with four weekly intralesional injections of triamcinolone. However, the patient did not respond and the lesions kept on increasing in size and number. The patient reported an episode of bleeding from the lesion, which led us to reconsider the diagnosis. Dermoscopy revealed white–pink structureless areas, irregular white lines with interspersed red clods, serpentine vessels and white scales [Figure 2a]. A recent lesion showed dilated vessels [Figure 2b] (DermLite DL4 × 10; 3 Gen, San Juan Capistrano, CA, USA).

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Figure 1:

Multiple, polysized, erythematous to hyperpigmented papules and nodules on the nape of neck.

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Figure 2:

(a) Dermoscopy reveals white pink structureless areas (yellow star), irregular white lines with interspersed red clods (blue star), serpentine and dotted vessels (red star) and white scales (black star). Red clods indicate vascular dilation (DermLite DL4 × 10; 3 Gen, San Juan Capistrano, CA, USA). (b) Presence of dilated vessel in recent onset lesion. (DermLite DL4 × 10; 3 Gen, San Juan Capistrano, CA, USA).

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HISTOPATHOLOGICAL FINDINGS

His peripheral eosinophil count and immunoglobulin E levels were normal. Excision biopsy revealed dilated blood vessels in the dermis with endothelial cells appearing as epithelioid cells in a background of multiple eosinophils and lymphocytes [Figure 3].

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Figure 3:

Dilated blood vessels in dermis with endothelial cells appearing as epithelioid cell (yellow star) and presence of plenty of eosinophils and lymphocytes (red star) (H and E, ×40). H&E: Haematoxylin and eosin.

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DIAGNOSIS

Based on clinical, dermoscopic and histopathological findings, the patient was diagnosed as a case of ALHE.

DISCUSSION

Nuchal papules are a common clinical presentation in the dermatology outpatient department, with AKN being the most common culprit. However, other entities such as folliculitis decalvans, hidradenitis suppurativa, dissecting cellulitis, acne conglobata, ALHE, pyogenic granuloma, follicular cutaneous T-cell lymphoma and bacterial folliculitis may mimic AKN and confound the clinician. Our case was diagnosed on the basis of dermoscopy and histopathology.

ALHE was first described in 1969 by Wells and Whimster.^[1] At present, ALHE also known as epithelioid haemangioma has been classified as a benign vascular tumour by the International Society for the Study of Vascular Anomalies in 2018. The tumour is very rare in India; however, its exact incidence or prevalence has not been reported. It usually presents with papules or nodules on the head, especially around the ears.^[2] Lesions limited to the nape of neck have been rarely reported. ALHE lesions represent localised proliferations of capillaries around medium-sized vessels with an epithelioid endothelial cell lining and are surrounded by associated lymphocytic and eosinophilic infiltrates as seen in our case.^[3]

ALHE can imitate multiple conditions, thereby leading to a diagnostic difficulty. Kimura’s disease is another entity that was earlier considered to be closely related to ALHE, but now, it is widely recognised that both are separate entities [Table 1].^[4-6] Although surgical excision is the treatment of choice, other modalities such as cryotherapy, electrofulguration, imiquimod and retinoids have been tried with success.^[6,7] Our patient underwent serial surgical excision with no relapse over a 6-month follow-up.

The take home message from this case includes:

1. ALHE also known as epithelioid haemangioma is a rare benign vascular tumour

2. Clinically, it may mimic AKN and pose a diagnostic difficulty

3. History of bleeding from any lesion should alert the treating doctor regarding the presence of vascular tumour or malformation

4. Dermoscopy is an important tool in diagnosing vascular tumours/malformation

5. Histopathologically ALHE is characterised by endothelial cells appearing as epithelioid cells in a background of multiple eosinophils.