|LETTER TO EDITOR
|Year : 2021 | Volume
| Issue : 2 | Page : 97-98
Neutrophilic eccrine hidradenitis presenting in a case of acute undifferentiated leukemia before chemotherapy
Akash Agarwal1, Priyanka Samal2, Debahuti Mohapatra3, Bikash Ranjan Kar1
1 Department of Dermatology, IMS and SUM Hospital, Bhubaneswar, Odisha, India
2 Department of Clinical Haematology, IMS and SUM Hospital, Bhubaneswar, Odisha, India
3 Department of Pathology, IMS and SUM Hospital, Bhubaneswar, Odisha, India
|Date of Submission||03-Oct-2020|
|Date of Decision||04-Apr-2021|
|Date of Acceptance||02-Jun-2021|
|Date of Web Publication||14-Dec-2021|
Department of Dermatology, IMS and SUM Hospital, K-8 Kalinga Nagar, Bhubaneshwar - 751 003, Odisha.
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Agarwal A, Samal P, Mohapatra D, Kar BR. Neutrophilic eccrine hidradenitis presenting in a case of acute undifferentiated leukemia before chemotherapy. Indian J Drugs Dermatol 2021;7:97-8
|How to cite this URL:|
Agarwal A, Samal P, Mohapatra D, Kar BR. Neutrophilic eccrine hidradenitis presenting in a case of acute undifferentiated leukemia before chemotherapy. Indian J Drugs Dermatol [serial online] 2021 [cited 2022 Jun 26];7:97-8. Available from: https://www.ijdd.in/text.asp?2021/7/2/97/332427
A 12-year-old boy was referred from the department of clinical hematology with a history of sudden eruption of multiple asymptomatic red elevated skin lesions for 5 days. There was no drug intake, insect bite, or similar episodes in the past. On cutaneous examination, multiple slightly tender, erythematous to hyperpigmented, succulent papules of varying sizes were distributed along the trunks, arms, and legs sparing the face [Figure 1] and [Figure 2]. The boy had been admitted for the evaluation of high-grade fever, sudden onset bilateral paraparesis with severe pallor, and hepatosplenomegaly. Laboratory data revealed pancytopenia (hemoglobin: 6.6%, white blood cell: 480/mm3; and platelets: 23,000/mm3) with peripheral smear showing blasts (5 out of 15 cells), which pointed toward an underlying hematological malignancy. Bone marrow aspiration and biopsy reports were awaited. Empirical broad-spectrum antibiotics were started in view of suspected sepsis.
|Figure 1: Multiple erythematous edematous succulent papules distributed on bilateral upper limb|
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|Figure 2: Close up view of right forearm illustrating the edematous erythematous papules|
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A punch biopsy from a representative lesion on the arm was performed with the differential diagnosis of Sweet’s syndrome, leukemia cutis, exaggerated insect bite reaction, and neutrophilic eccrine hidradenitis (NEH). Histological findings revealed dense mixed perieccrine inflammatory infiltrate comprised mostly of neutrophils, with few plasma cells, lymphocytes, and mononuclear cells. Vacuolar degeneration and necrosis of eccrine glands characteristic of NEH were present [Figure 3] and [Figure 4]. Tissue cultures for bacteria and fungi were negative. The absence of dense dermal neutrophilic infiltrate, atypical lymphocytes, and eosinophilic perivascular infiltrate with red blood cell dropouts ruled out the other differential diagnosis, respectively.
|Figure 3: Histopathology shows normal epidermis with focal infiltration seen around the eccrine glands (H and E, ×4)|
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|Figure 4: Histopathology shows eccrine gland infiltrated by predominant neutrophilic inflammatory infiltrate with focal necrosis and vacuolar degeneration (black arrow). Neutrophils around eccrine gland can be visualized (red arrow) (H and E, ×40)|
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Bone marrow aspiration and biopsy reports confirmed diagnosis of acute leukemia with 64% blasts and myeloperoxidase negativity. Flow cytometry further classified leukemia as acute undifferentiated type. Lumbar puncture revealed blasts in cerebrospinal fluid (CSF) which explained the paraparesis. Blood culture grew Staphylococcus aureus sensitive to teicoplanin. A final diagnosis of NEH with acute undifferentiated leukemia and paraparesis was established. Given the benign self-limiting nature of NEH and underlying sepsis, only topical steroids were given. During the hospital stay of 2 weeks, most of the lesions flattened with postinflammatory hyperpigmentation.
NEH is a rare entity which was first described by Harris et al. in 1982 in a patient of acute myeloid leukemia (AML) receiving induction chemotherapy. In 90% of cases, it is associated with chemotherapy (bleomycin and cytarabine) in AML patients, but other malignancies are also known to be associated. NEH has also rarely been reported in patients with bacterial infections, Behcet’s disease, HIV, and in those receiving GM-CSF treatment. In addition, it has been described in otherwise healthy children during the summer season.
The histological diagnosis of NEH in our patient was made before the identification and confirmation of underlying leukemia, hence before any possible chemotherapeutic intervention. Infection as a cause for NEH can be considered given the presentation of patient with sepsis. However, since tissue culture was negative, and minimal to no response was seen in skin lesions after broad-spectrum antibiotics, the likelihood of this is mitigated. To our knowledge, only four similar cases have been reported in literature. In two reports, NEH heralded the onset of AML., In other two cases, NEH developed when the patient suffered a relapse of AML at least 6 months for last chemotherapy cycle., Pierson et al. had suggested that perhaps a subset of NEH patients might have paraneoplastic eccrine changes. The underlying pathomechanism however is not clearly understood.
Acute undifferentiated leukemia in pediatric age group is rare with more than 70% of patients being 60 years or older. NEH heralding the onset of acute undifferentiated leukemia in pediatric age group has not been previously described in literature. Through our report, we would like to add to the understanding that NEH may possibly represent a paraneoplastic phenomenon as in Sweet’s syndrome. The report also adds to the claim by many authors that NEH must be considered under the spectrum of neutrophilic dermatoses.
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], [Figure 3], [Figure 4]