|Year : 2020 | Volume
| Issue : 4 | Page : 323-325
Subcorneal pustular dermatosis: An unusual presentation in pediatric age group
Shivani Saini, Devendra Yadav, Akshy Kumar
Department of Skin and VD, GMC, Kota, Rajasthan, India
|Date of Submission||15-Feb-2020|
|Date of Decision||16-Mar-2020|
|Date of Acceptance||26-Apr-2020|
|Date of Web Publication||30-Sep-2020|
Dr. Akshy Kumar
Department of Skin and VD, GMC, Kota, Rajasthan
Source of Support: None, Conflict of Interest: None
Subcorneal pustular dermatosis (SPD), first described by Sneddon and Wilkinson, represents a rare chronic and relapsing sterile pustular eruptions generally involving the trunk and flexor aspect of limbs. Usually, it affects middle-aged or elderly women. Histologically, it is characterized by the accumulation of subcorneal neutrophils with an absence of spongiosis and acantholysis. Here, we are reporting a rare case of SPD in the pediatric age group, which was diagnosed based on clinical and histological findings. We gave dapsone in treatment with a positive outcome. To the best of our knowledge, there has not been any case report in the pediatric age group from India so far.
Keywords: Dapsone, pediatric, sterile pustular eruption, subcorneal neutrophils, subcorneal pustular dermatosis
|How to cite this article:|
Saini S, Yadav D, Kumar A. Subcorneal pustular dermatosis: An unusual presentation in pediatric age group. Indian J Paediatr Dermatol 2020;21:323-5
|How to cite this URL:|
Saini S, Yadav D, Kumar A. Subcorneal pustular dermatosis: An unusual presentation in pediatric age group. Indian J Paediatr Dermatol [serial online] 2020 [cited 2020 Oct 22];21:323-5. Available from: https://www.ijpd.in/text.asp?2020/21/4/323/296854
| Introduction|| |
Subcorneal pustular dermatosis (SPD), also known as Sneddon–Wilkinson disease, is an uncommon, benign, chronic, relapsing, pustular eruption first described by Sneddon and Wilkinson. It is occasionally classified under the category of neutrophilic dermatosis with predominantly trunk and proximal flexural involvement. It most frequently affects adults of 40–60 years of age with the female-to-male ratio of about 4:1, but rarely, may present in childhood. Histologically, it is characterized by the subcorneal accumulation of neutrophils with the absence of spongiosis and acantholysis, a hallmark of this condition.
| Case Report|| |
A 16-year-old male child presented to the dermatology outpatient department with the 1-month-old itchy, pus-filled eruptions of variable size, located on the trunk predominantly, below axillary area, and on the buttocks. The classic lesion was presented as a “half and half” blister, also known as hypopyon sign, in which purulent fluid seemed to settle at the base of the blister. The patient reported that some blisters had developed within a few hours. He had a history of atopic dermatitis with no family history of any of the diseases.
Physical examination revealed multiple grouped flaccid pustules of varying sizes from 2 to 10 mm, some got coalesced to form annular, circinate, and serpiginous lesions in pattern and superficial crusts on the normal or mildly erythematous skin, over the trunk, infraaxillary area and on the buttocks. Hypopyon sign was seen in some lesions. Residual hyperpigmentation was there, indicating healed lesions [Figure 1]a, [Figure 1]b, [Figure 1]c. All the relevant laboratory data were collected, which included complete blood count, erythrocyte sedimentation rate, serum electrolytes, blood urea nitrogen, serum creatinine, liver function tests, urinalysis, and chest radiographs. Skin biopsy from the intact pustule was performed and submitted for the hematoxylin and eosin staining. Histopathology demonstrated subcorneal pustule just below the stratum corneum and contained many neutrophils with few eosinophils. In the dermis, superficial blood vessels were surrounded by a nonspecific mixed inflammatory cell infiltrate consisting mainly of neutrophils [Figure 2]a and [Figure 2]b. Direct immunofluorescence (DIF) was done to rule out other immunobullous disorders, and it was found negative for immunoglobulin A (IgA) intercellular accumulation [Figure 2]c.
|Figure 1: Multiple grouped flaccid pustular lesions present over trunk, infraaxillary area and buttocks, some forming annular, circinate, and serpiginous pattern (a-c before treatment)|
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|Figure 2: Histopathology shows subcorneal accumulation of neutrophils without spongiosis and acantholysis with non-specific mixed inflammatory cells in dermis (H and E stain, ×4 [a], ×10x [b]), direct immunofluorescence was negative for immunoglobulin A accumulation (c)|
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Based on the clinical and histopathological findings, a diagnosis of SPD was made. After normal G-6PD estimation, our patient was being prescribed dapsone 100 mg in a day orally and got dramatic relief in his disease within 4 weeks [Figure 3]a and [Figure 3]b. After complete clinical remission, the dose tapered to 50 mg/day.
|Figure 3: (a and b) Moderate improvement in lesions at 4 weeks after giving treatment|
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| Discussion|| |
The etiology of the disease is not fully understood to date. There are some reports of subcorneal pustular dermatosis (SCPD) in association with benign monoclonal IgA gammopathy, synovitis–acne–pustulosis–hyperostosis–osteitis syndrome, Sjogren's syndrome, and rheumatoid arthritis.
The disease involves more commonly trunk, intertriginous areas and flexor aspects of the limbs; the face is involved very rarely, as in our case. Some cases reported pustules on the palms and soles. Mucous membranes are seldom affected.
In our case, the history, physical examination, and relevant laboratory investigations did not reveal any association with systemic diseases.
The differential diagnoses of SCPD include impetigo, pustular psoriasis, dermatophyte infections, immunobullous diseases (linear IgA disease, pemphigus, dermatitis herpetiformis, and intercellular IgA diseases). For dermatophyte infections, direct microscopic examination for fungal elements would reveal the diagnosis. Nails and scalp are uncommonly affected in SCPD, unlike in pustular psoriasis, and also spongiform pustules, microabscess, and elongation of rete ridges do not occur in classical SCPD. Patients usually have fever and leukocytosis in the case of generalized pustular psoriasis. IgA deposition is found in the dermal papillae in the case of dermatitis herpetiformis. In the case of IgA pemphigus, the acantholysis is more pronounced than in SCPD; moreover, DIF studies demonstrate intercellular IgA deposition in squamous cells.
Dapsone remains the treatment of choice. Although it is an uncommon condition in childhood still very few cases have been reported [Table 1].
|Table 1: Summary of some reported pediatric cases of subcorneal pustular dermatosis|
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Our case is worth reporting as rare because there is no pediatric case report from India to date.
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.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]