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E-IJD® - CORRESPONDENCE
Year : 2022  |  Volume : 67  |  Issue : 4  |  Page : 481
Pemphigus foliaceous with prominent neutrophilic pustules: A rare variant responsive to colchicine


1 From the Department of Dermatology, Venereology and Leprosy, ABVIMS and Dr. Ram Manohar Lohia Hospital, New Delhi, India
2 Department of Pathology, ABVIMS and Dr. Ram Manohar Lohia Hospital, New Delhi, India

Date of Web Publication2-Nov-2022

Correspondence Address:
Sinu Rose Mathachan
From the Department of Dermatology, Venereology and Leprosy, ABVIMS and Dr. Ram Manohar Lohia Hospital, New Delhi
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijd.ijd_672_21

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How to cite this article:
Mathachan SR, Arora P, Sardana K, Paliwal P. Pemphigus foliaceous with prominent neutrophilic pustules: A rare variant responsive to colchicine. Indian J Dermatol 2022;67:481

How to cite this URL:
Mathachan SR, Arora P, Sardana K, Paliwal P. Pemphigus foliaceous with prominent neutrophilic pustules: A rare variant responsive to colchicine. Indian J Dermatol [serial online] 2022 [cited 2022 Dec 4];67:481. Available from: https://www.e-ijd.org/text.asp?2022/67/4/481/360348




Sir,

A 15-year-old male patient presented with generalized erythema, crusting, scaling (yellowish to dirty brown), and erosions over the body, gradually progressing into erythroderma [Figure 1]a and [Figure 1]b. During the course of admission, he developed new flaccid fluid-filled vesicles/bulla over upper and lower limbs with a positive direct and marginal Nikolsky sign. Potassium hydroxide (KOH) mount and gram stain from scales and erosions, respectively, were negative for fungal, mite, and bacterial infection. Tzanck smear showed acantholytic cells. Skin biopsy was done from an intact vesicle and revealed focal parakeratosis, subcorneal cleft containing a few neutrophils and acantholytic cells, irregular acanthosis, neutrophilic exocytosis, and spongiosis along with moderate perivascular mixed inflammatory infiltrate in the dermis. DIF (Direct immunofluorescence) showed IgG and C3 deposits on the intercellular surface of keratinocytes predominantly located at the stratum granulosum [Figure 2]a and [Figure 2]b. Based on clinical and laboratory evaluation, a diagnosis of erythroderma secondary to pemphigus foliaceous (PF) was made. Treatment was initiated with prednisolone 30 mg and dapsone 100 mg once daily. After 2 weeks, the dose of prednisolone was hiked to 35 mg and azathioprine 50 mg twice daily was added owing to lack of improvement and appearance of new lesions.
Figure 1: (a) The trunk showed extensive erythema, few erosions, and crusting. (b) Crusted erosions over the chest, both upper limbs involving hands and fingers. (c) Discrete and coalescing pinhead sized pustules over the thigh and knee joint. (d) Few pustules coalesced over the buttocks to form lakes of pus along with extensive yellowish crusts.

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Figure 2: (a) Histopathology showed sub corneal cleft containing a few neutrophils and acantholytic cells, irregular acanthosis, and spongiosis (H&E 200×). (b) Direct immunofluorescence showed IgG and C3 deposits on the intercellular surface of keratinocytes predominantly located at the stratum granulosum (DIF 10×). (c) Skin biopsy showing subcorneal collection of neutrophils (black arrow) and subcorneal acantholytic cells (red arrow), in an acanthotic spongiotic epidermis (H&E × 400).

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Three weeks later, the patient started developing discrete and coalescing pinhead-sized pustules predominantly over the trunk, buttocks, along the nail folds, and subungual area [Figure 1]c and [Figure 1]d. Few pustules coalesced over certain areas like buttocks and thighs to form lakes of pus. In view of the change in the clinical picture, the skin biopsy was repeated with provisional diagnosis of pustular psoriasis, SCPD, IgA pemphigus, and pemphigus foliaceous with pustules. Gram stain done from the pustular lesion was sterile, and skin biopsy showed a subcorneal collection of neutrophils and acantholytic cells in an acanthotic spongiotic epidermis [Figure 2]c. DIF was repeated and showed with IgG and C3 positivity on the intercellular surface of keratinocytes, and IgA was negative. Immunoblot and enzyme-linked immunosorbent assay (ELISA) could not be done due to financial constraints. A final diagnosis of PF with prominent neutrophilic pustules was made. Dapsone and azathioprine were stopped after 8 weeks due to lack of response, and tablet colchicine 0.5 mg three times a day was added along with prednisolone 30 mg daily. The pustules resolved gradually, and erythema and scaling decreased significantly. Colchicine and prednisolone were continued for another 1 month with gradual tapering of the dose. The patient was in remission for a period of 6 months.

Pemphigus foliaceous has several variants like pemphigus erythematosus, pemphigus herpetiformis, and endemic PF. Pemphigus erythematosus is typically characterized by erythematous scaly lesions in a butterfly distribution over the nose and cheeks similar to that of lupus erythematosus. The DIF will show the fish-net pattern of intercellular IgG along with a linear deposit of IgG and/or C3 at the dermo-epidermal junction. Pemphigus herpetiformis clinically resembles dermatitis herpetiformis characterized by clusters of pruritic papules and vesicles on an erythematous background with variable acantholysis and eosinophilic/neutrophilic spongiosis on histopathology. While endemic PF is a Brazilian variant of PF that is believed to be caused by black fly (Simulium prurinosum) with a striking distribution of lesions mainly on the photo exposed skin.

PF with neutrophilic pustules is a rare presentation, with only a few cases been reported in literature.[1],[2],[3],[4] All previously reported cases presented as erythroderma with extensive skin involvement similar to our patient. Clinically, the presence of predominant pustules in our patient was mimicking pustular psoriasis/IgA pemphigus/subcorneal pustular dermatosis (SCPD). However, flexures were spared, characteristic serpiginous/annular pattern of SCPD/IgA pemphigus was not seen, and the patient had no constitutional symptoms like fever/myalgia in favor of pustular psoriasis. Another rare subset of pemphigus with a similar presentation is IgA/IgG pemphigus with DIF showing anti-keratinocyte cell surface antibodies of both IgG and IgA classes.[5] DIF in our case was negative for IgA deposits, thereby ruling out both IgA pemphigus and IgG/IgA pemphigus. We also noted treatment refractoriness to steroids and immunosuppressive agents in our case in contrast to classical PF, which usually responds well to these treatments. Matsuo et al.[1] and Méndez-Flores et al.[2] also reported frequent relapses and incomplete resolution on treatment (oral prednisolone and dapsone) in their cases who presented as erythrodermic PF with neutrophilic pustules. However, the addition of colchicine, along with steroids in our case, showed remarkable improvement and resolution of pustules in 2 weeks.

The cause for the neutrophilic infiltration in PF is unclear; however, studies have found a key role of IL-8 for neutrophil chemotaxis and migration to epidermis.[3] It is also reported that the role of complement in pustule formation is negligible.[4] Colchicine is a drug that preferentially concentrates in neutrophils. We assume that the prompt resolution of pustular eruption in our case could be due to the diverse mechanism of action of colchicineas described in [Table 1].[6] Apart from its action against neutrophils, it also has anti-inflammatory and immunosuppressive function adding to the synergistic effects of oral steroids in our case.
Table 1: Table elaborating the diverse mechanism of action of colchicine and its possible role in our case[6]

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To conclude, we highlight the unusual association of neutrophilic pustules and PF and the favorable outcome of colchicine in this rare variant of PF not responding to the conventional treatment modalities.

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 Top

1.
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.  Back to cited text no. 1
    
2.
Méndez-Flores S, Avalos-Diaz E, Dominguez-Cherit J, Saeb-Lima M, Esquivel-Pedraza L. Pemphigus foliaceus with circinated plaques and neutrophil pustules. J CutanPathol2016;43:1062-6.  Back to cited text no. 2
    
3.
O'toole EA, Mak LL, Guitart J, Woodley DT, Hashimoto T, Amagai M, et al. Induction of keratinocyteIL-8 expression and secretion by IgG autoantibodies as anovel mechanism of epidermal neutrophil recruitment ina pemphigus variant. Clin Exp Immunol 2000;119:217-24.  Back to cited text no. 3
    
4.
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.  Back to cited text no. 4
    
5.
Kanwar AJ, Vinay K, Saikia UN, Koga H, Teye K, Tsuruta D, et al. IgG/IgA pemphigus reactive with desmoglein 1 with additional undetermined reactivity with epidermal basement membrane zone. Indian J Dermatol VenereolLeprol 2014;80:46-50.  Back to cited text no. 5
    
6.
Sardana K, Sinha S, Sachdeva S. Colchicine in dermatology: Rediscovering an old drug with novel uses. Indian Dermatol Online J2020;11:693-700.  Back to cited text no. 6
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