|Year : 2021 | Volume
| Issue : 2 | Page : 240-241
Multiple itchy nodules over the scalp – clinicopathological challenge
Aditi Dhanta, Gargi Taneja, Neirita Hazarika, Michael Leonald Anthony
Department of Dermatology, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India
|Date of Submission||01-Jun-2020|
|Date of Decision||07-Sep-2020|
|Date of Acceptance||23-Nov-2020|
|Date of Web Publication||26-Aug-2021|
Department of Dermatology, All India Institute of Medical Sciences, Rishikesh, Uttarakhand
Source of Support: None, Conflict of Interest: None
Angiolymphoid hyperplasia with eosinophilia is an uncommon benign vascular proliferation with histologically distinct endothelial cells and a mixed inflammatory infiltrate composed of mainly lymphocytes and eosinophils unknown. It commonly affects middle-aged adults and characterized by flesh/plum-colored pruritic nodules and papules. In our case, it has to be differentiated from other appendageal tumors such as cylindroma, spiradenoma because of similar site involvement. The histopathological examination is a fundamental tool to achieve a correct diagnosis.
Keywords: Angiolymphoid hyperplasia with eosinophilia, nodules, plump endothelial cells
|How to cite this article:|
Dhanta A, Taneja G, Hazarika N, Anthony ML. Multiple itchy nodules over the scalp – clinicopathological challenge. Clin Dermatol Rev 2021;5:240-1
| Introduction|| |
Angiolymphoid hyperplasia with eosinophilia (ALHE) is an uncommon benign vascular neoplasm of debated etiology. It commonly occurs on the head and neck, especially at ear and periauricular area, and has a tendency to recur after excision. It usually manifests as solitary or multiple erythematous to brownish papules or nodules. We describe here a distinctive case with regard to the location and gross appearance of the tumor.
| Case Report|| |
A 40-year-old female presented with multiple itchy reddish nodules on her right side of the scalp, gradually increasing in number and size over a period of 6 months. There was no history of preceding trauma or surgery at the site. There was no history of bleeding, but the patient complained of severe pruritus in the lesions. Cutaneous examination revealed multiple firms, nontender, well-defined erythematous to skin-colored papules, and nodules of varying size 0.5 cm × 0.5 cm to 1.5 cm × 1.5 cm in diameter, present discrete or in groups in the right side of the occipital and parietal region of the scalp [Figure 1]a. Few nodules were surmounted by erosions and crusting [Figure 1]b. There was no regional lymphadenopathy and the rest of the systemic examination was normal.
|Figure 1: (a) Multiple well-defined erythematous papules and nodules of varying size 0.5 cm × 0.5 cm to 1.5 cm × 1.5 cm with erosions over nodules in the right side of the occipital region of the scalp. (b) Multiple well-defined erythematous papules and nodules of varying size 0.5 cm × 0.5 cm to 1.5 cm × 1.5 cm with erosions over nodules in the right side of the parietal region of the scalp|
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Routine laboratory investigations including a complete hemogram, hepatic profile, renal profile, and immunoglobulin E (IgE) levels were done. Eosinophilia of 18% was notable while other parameters were within normal limits. A 4-mm punch biopsy was taken from the nodule for histopathological evaluation. Microscopy revealed multiple well-defined collections of inflammatory infiltrate composed of lymphocytes and eosinophils with dilated vascular channels present in the upper, mid, and lower dermis with multiple pilosebaceous units. The vascular channels lined by plump, partly vacuolated endothelial cells. They are epitheloid in appearance and protrude into the vessels lumen with a hobnail-like appearance [Figure 2]a, [Figure 2]b, [Figure 2]c. Based on the characteristic histopathological features, a diagnosis of ALHE was established.
|Figure 2: (a) Multiple well-defined collection of inflammatory infiltrate with dilated vascular channels present in upper, mid, and lower dermis with multiple pilosebaceous units (H and E, ×4). (b) Collection of inflammatory infiltrates composed of lymphocytes, eosinophils present in the fibrous stroma with dilated blood vessels containing plump endothelial cells (H and E, ×10). (c) On high power collection of inflammatory cells is composed of lymphocytes and eosinophils in the stroma. The vascular channels are lined by plump, partly vacuolated endothelial cells. They are epitheloid in appearance and protrude into the vessels lumen with a hobnail-like appearance present within a fibrotic stroma (H and E, ×40)|
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| Discussion|| |
The term ALHE was first described by Wells and Whimster in 1969. The exact etiopathogenesis of ALHE is unknown, however, various hypotheses have been proposed, including environmental factors such as insect bite or parasites, trauma, hyperestrogenic state, and immunological mechanism. Some authors suggested it may be a reactive process or a neoplastic process; however, none have proven to be conclusive or definitive. ALHE occurs in the third to fourth decade of life, with a female preponderance. It present as an erythematous or skin-colored dome-shaped dermal papules or nodules on the head and neck, especially on the ears and periauricular region. It is often associated with spontaneous bleeding, pain, and pruritus. Eosinophilia is evident in only 20% of ALHE cases and elevated IgE levels and regional lymphadenopathy is a rare finding.
Its main differential diagnosis is kimura's disease but a variety of vascular tumors such as angiomatous lymphoid hamartoma, hemangioma, pyogenic granuloma, kaposi sarcoma, lymphoma, angiosarcoma, and epithelioid hemangioendothelioma may resemble clinically to ALHE. ALHE should be differentiated from Kimura's disease which is characterized by a triad of subcutaneous masses in the head-and-neck region, peripheral eosinophilia, and elevated serum IgE levels while histopathology shows follicular hyperplasia, reactive germinal centers, and the presence of eosinophilic infiltrates in the interfollicular areas. Furthermore, the presence of lymphadenopathy points more toward Kimura's disease.
The confirmation of the diagnosis is made by histopathology. Characteristic histopathological features are the presence of epithelioid endothelial cells lining the vascular wall and projecting inside the vascular lumen giving a “cobblestone” appearance. These cells have large nuclei and abundant eosinophilic cytoplasm. A perivascular inflammatory cell infiltrate composed mainly of lymphocytes, and eosinophils are second defining characteristic of ALHE.
ALHE is a benign disease but can be resistant to multiple therapies, and can become disfiguring to the patient. Surgical excision is the treatment of choice and when completely excised, it rarely recurs. One-third of cases recur after incomplete excision. Other treatment modalities used to treat ALHE with variable response, including intralesional corticosteroids, cryosurgery, electrodesiccation, topical tacrolimus, interferon alfa-2a, topical imiquimod, pentoxyphilline, and oral retinoids.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]