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CORRESPONDENCE |
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| Year : 2022 | Volume
: 67
| Issue : 6 | Page : 805-807 |
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| Pemphigus foliaceous presents as annular pustular lesion: A diagnostic confusion |
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Abhijit Saha1, Subhra Dhar2
1 From the Department of Pediatric Dermatology, Institute of Child Health, Kolkata, West Bengal, India
2 Wizderm Path Lab, Kolkata, West Bengal, India
| Date of Web Publication | 23-Feb-2023 |
Correspondence Address:
Abhijit Saha
Department of Pediatric Dermatology, Institute of Child Health, Kolkata, West Bengal
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/ijd.ijd_247_22
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How to cite this article:
Saha A, Dhar S. Pemphigus foliaceous presents as annular pustular lesion: A diagnostic confusion. Indian J Dermatol 2022;67:805-7 |
Sir,
Pemphigus foliaceous (PF) is a chronic autoimmune blistering disorder where immunoglobulin G (IgG) deposits on the cell surface of epidermal keratinocytes against desmoglein-1, a transmembrane constituent of cell adhesion molecule desmosome.
We report a case of a 65-year-old woman who presented with erythematous, scaly, annular, pruritic lesions almost all over the body excluding the palms, soles, face, and scalp. On clinical suspicion of superficial fungal infection, oral anti-fungal was administered. Follow-up after three weeks revealed no improvement. Surprisingly, we noticed many fresh annular lesions with peripheral superficial pustules [Figure 1]. Her scalp and face were involved this time with erosion, crust, and erythema. We considered few other differentials like pemphigus foliaceous (PF), subcorneal pustular dermatoses (SPD), IgA pemphigus, generalized pustular psoriasis (GPP), and acute generalized exanthematous pustulosis (AGEP). We took two 3-mm punch biopsy: one from periphery of an annular lesion and another intact vesicle for direct immunofluorescence (DIF) and histopathology examination, respectively. Histopathology revealed superficially eroded upper epidermis with few attached acantholytic cells [Figure 2]. Few eosinophils were seen within the epidermis. The dermis showed mixed inflammatory infiltrate. DIF showed intercellular deposition of IgG and C3 [Figure 3]. DIF was negative for IgA. | Figure 2: (H and E 100×): Histopathology revealed superficially eroded upper epidermis with few attached acantholytic cells and eosinophils. White arrow denotes acantholytic cell and red arrow denotes two eosinophil side by side at the level of upper spinous layer
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 | Figure 3: (DIF): DIF showed intercellular deposition of IgG and C3 (white arrow) at the level of upper spinous layer
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Constellation of clinical, histopathology, and DIF findings helped us to arrive at a diagnosis of PF.
This kind of presentation of pemphigus foliaceus poses a diagnostic challenge to the physician. Clinically, IgA pemphigus presents with vesico-pustular lesions over normal or erythematous base in flexural area like the axillae and the groin. Lesions have a tendency to coalesce to form annular plaque. On histopathological examination, SPD type of IgA pemphigus showed sub-corneal neutrophilic pustules with minimal acantholysis. Intra-epidermal neutrophilic (IEN) type demonstrated supra-basal cleavage. Intense epidermal neutrophilic infiltration is a rule in IgA pemphigus. DIF demonstrated intercellular deposits of IgA on keratinocyte cell surface. SPD though clinically and histologically is akmost indistinguisable from Ig A pemphigus, DIF is typically negetive for SPD. GPP is an uncommon variant of psoriasis which presents with sterile pustules on an erythematous background with systemic symptoms. Along with classical histological features of psoriasis vulgaris, presence of spongiform pustules of kogoj in the upper epidermis clinches the diagnosis. Following exposure to the culprit drug, AGEP clinically presents with non-follicular sterile pustules on an erythematous oedematous background with fever. Eruption usually starts from the face and gradually involves the trunk and lower extremities. Subcorneal pustules is the most important histological finding. Neutrophilic leukocytosis is frequently seen.
PF presenting as annular erythematous lesion with peripheral pustules has rarely been reported, to the best of our knowledge. Polycyclic pattern has been described in childhood sporadic PF.[1] Though neutrophilic pustules are rare in PF, a few reports described the same.[2],[3] Most available reports mentioned association of PF with GPP or annular pustular psoriasis (APP).[4] Several hypotheses have been postulated to explain the presence of pustules in PF, including drug and epitope, among which the most important consideration is probably elevated level of interleukin (IL)-8 which acts as a chemoattractant for neutrophil recruitment.[2],[3],[4],[5] Though we did not find any neutrophil within this particular histopathology section, it does not disprove the possible mechanism of pustule formation discussed before.
Proper awareness about this unusual clinical presentation of PF is necessary for prompt diagnosis and treatment.
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
Nil.
Conflicts of interest
There are no conflicts of interest.
 References | |  |
| 1. | Metry DW, Hebert AA, Jordon RE. Nonendemic pemphigus foliaceus in children. J Am Acad Dermatol 2002;46:41922.  |
| 2. | Miyakura T, Yamamoto T, Okubo Y, Ishii N, Oyama B, Hashimoto T, et al. Pemphigus foliaceus with prominent neutrophilic pustules initially presenting as erythroderma. Clin Exp Dermatol 2009;34:e46-9. |
| 3. | Matsuo K, Komai A, Ishii K, Futei Y, Amagai M, Deguchi H, et al. Pemphigus foliaceus with prominent neutrophilic pustules. Br J Dermatol 2001;145:132-6. |
| 4. | Kato K, Hanafusa T, Igawa K, Tatsumi M, Takahashi Y, Yamanaka T, et al. A rare case of annular pustular psoriasis associated with pemphigus foliaceus. Ann Dermatol 2014;26:260-1. |
| 5. | Mendez-Flores S, Dominguez-Cherit J, Garcia-Hidalgo L, Saeb-Lima M. A rare presentation of pemphigus foliaceus with prominent neutrophilic Pustules. J Am Acad Dermatol 2012;66:132-6. |
[Figure 1], [Figure 2], [Figure 3] |
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