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| Year : 2022 | Volume
: 67
| Issue : 6 | Page : 829-831 |
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| Multiple pruritic keratotic papules on the extremities in a 14-Year-Old Boy |
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Sahana M Srinivas1, Sandipan Dhar2, Subhra Dhar3, Rashmi Agarwal4
1 From the Consultant Pediatric Dermatologist, Department of Pediatric Dermatology, Indira Gandhi Institute of Child Health, Bengaluru, Karnataka, India
2 Department of Pediatric Dermatology Institute of Child Health, Kolkata, West Bengal, India
3 Consultant Pathologist, WIZDERM Path Lab, Kolkata, West Bengal, India
4 Consultant Pediatric Dermatologist, Cutis Academy of Cutaneous Sciences, Bengaluru, Karnataka, India
| Date of Web Publication | 23-Feb-2023 |
Correspondence Address:
Sahana M Srinivas
Department of Pediatric Dermatology, Indira Gandhi Institute of Child Health, Bengaluru, Karnataka
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/ijd.ijd_222_21
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How to cite this article:
Srinivas SM, Dhar S, Dhar S, Agarwal R. Multiple pruritic keratotic papules on the extremities in a 14-Year-Old Boy. Indian J Dermatol 2022;67:829-31 |
How to cite this URL:
Srinivas SM, Dhar S, Dhar S, Agarwal R. Multiple pruritic keratotic papules on the extremities in a 14-Year-Old Boy. Indian J Dermatol [serial online] 2022 [cited 2023 Mar 23];67:829-31. Available from: https://www.e-ijd.org/text.asp?2022/67/6/829/370281 |
A 14-year-old boy presented with pruritic, hyperpigmented skin lesions on the extremities for 2 years. The lesions initially started as small blackish papules on the lower legs and slowly progressed to involve the forearms. These lesions were persistent. He was a known case of Wilson's disease on treatment with d-penicillamine for the past 3 years. The personal and family history was unremarkable. The general and systemic examinations were normal. On cutaneous examination, there were multiple, hyperpigmented, discrete, and grouped keratotic papules over the extensor aspect of both the lower legs and the right forearm [Figure 1]a and [Figure 1]b. Koebner phenomenon was seen on the lower legs. Generalized xerosis was present. The scalp, oral, genital mucosa, palms, and soles were normal. Routine hemogram, biochemistry profile, and urine analysis were normal. Dermoscopy showed polygonal to round-oval brownish-black pigmented areas surrounded by a whitish halo and streaks in a few areas with associated scaling. The surrounding areas showed multiple closely set yellowish-brown oval areas over an erythematous base with scaling [Figure 2]. A skin biopsy of one of the keratotic papules was performed. | Figure 1: (a) Multiple, hyperpigmented, discrete, and grouped keratotic papules on the extensor aspect of the lower leg. (b) Annular keratotic plaque with mild atrophy at the center on the right forearm.
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 | Figure 2: Polygonal to round-oval brownish-black pigmented areas surrounded by a whitish halo and streaks in a few areas with associated scaling (black arrow). The surrounding areas show multiple closely set yellowish-brown oval areas on an erythematous base with scaling (blue arrow)
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 What is the Diagnosis? | |  |
Persistent form of acantholytic dermatosis (Grover's disease).
| Histopathological Findings | | |
The epidermis showed hyperkeratosis, orthokeratosis, acanthosis, and irregular broad rete ridges. Small suprabasal clefts were seen along with scattered acantholytic keratinocytes and occasional apoptotic bodies. There was mild lymphohistiocytic infiltrate in the perivascular areas. Verhoef-van Gieson stain showed no increased or abnormal elastic fibers. The above findings simulated a histological pattern of Darier's disease [Figure 3]a and [Figure 3]b. | Figure 3: (a) The epidermis shows hyperkeratosis, orthokeratosis, acanthosis, and irregular broad rete ridges. Small suprabasal clefts were seen along with scattered acantholytic keratinocytes and occasional apoptotic bodies [H and E X100]. (b) A close-up view of the epidermis showing the suprabasal cleft [H and E X400]
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| Discussion | | |
Grover's disease, also known as transient acantholytic dermatosis (TAD), is an acquired, acantholytic disorder seen in elderly people, characterized by a papulovesicular eruption and histologically by focal acantholytic dyskeratosis.[1] It was first described by Ralph W. Grover in 1970, and later in 1977, Chalet et al.[2] established the diagnostic clinicopathological criteria of the disease. The term TAD is misleading as the skin lesions are often persistent for a few months to years or may be relapsing.[3] It is more commonly seen in middle-aged and elderly people and a few cases have been reported in children. The exact etiopathogenesis is still elusive, however, it has been postulated that poral occlusion or damaged intraepidermal ducts may lead to the leakage of molecules and acantholysis.[3] Various exacerbating factors include sweating, sunlight, heat exposure, ionizing and ultraviolet radiation, and prolonged hospitalization. In a systematic review of associated comorbidities with Grover's disease in adults, malignancies were the most commonly reported. The associated malignancies include leukemia, lymphoma, myelodysplastic syndromes, solid tumors of the genitourinary tract, prostate cancer, gastric carcinoma, and metastatic melanoma. It has occasionally been reported with post-renal and bone marrow transplant, chronic renal failure on hemodialysis, atopic dermatitis, HIV infection, and antineoplastic agents.[1],[4] Zvulunov A, et al.[5] reported an adult case of association of TAD with progressive systemic sclerosis treated with d-penicillamine therapy. The sulfhydryl groups of d-penicillamine are known to cause acantholysis by the disruption of disulfide bonds of cell adhesion molecules like desmogleins with subsequent dehiscence of desmosomes.[5] In our case, the possible etiology could be due to the d-penicillamine therapy.
Grover's disease can present as asymptomatic to pruritic edematous papules, papulovesicles, and rarely as keratotic papules. Less commonly pustules, bullae, erythematous plaque-like lesions, and nodules can be the presenting features. Linear, blaschkoid, zosteriform, and oral lesions resembling aphthae have been described in the literature.[6] Though truncal involvement is commonly seen, it can involve the upper and lower limbs, head, neck, hands, feet, and the vulval region. Three variants have been described based on the duration of the lesions: transient eruptive, chronic asymptomatic, and persistent pruritic. Differential diagnoses considered in our case were elastosis perforans serpiginosa, reactive perforating collagenosis, and Kyrle disease, but histopathology confirmed the case as Grover's disease. Histologically, Grover's disease resembles Darier's disease-like, Hailey-Hailey-like pattern, spongiotic pattern, pemphigus vulgaris, or pemphigus foliaceus-like pattern.[3] Our case showed Darier's disease-like histological pattern. Though papulovesicular lesions are characteristic of Grover's disease, the case presented here is of Darier's variant, hence, the characteristic lesion is keratotic papules. Older lesions become keratotic due to considerable acanthosis with subtle clefting and acantholysis.
The polygonal brownish-black pigmented areas seen on dermoscopy correspond to the compact hyperkeratosis while the white halo and streaks correspond to the suprabasal acantholysis with dyskeratosis and acanthosis. These findings are consistent with the previously reported dermoscopic findings of a Darier-like variant of Grover's disease (GD). Though similar findings can be present in Darier's disease also, these particular dermoscopic features can help in distinguishing it from other variants of Grover's disease and also other disease entities presenting in a similar fashion like perforating disorders.[7]
The prognosis is good as Grover's disease is self-limiting in nature. The avoidance of triggers, topical corticosteroids, keratolytics, and calcipotriol is helpful in mild disease. Systemic therapies that have been tried for severe and persistent form include low-dose systemic corticosteroids, retinoids, dapsone, tetracyclines, phototherapy, methotrexate cyclosporine, etanercept, rituximab, and total skin electron beam therapy.[1] The boy was started only on emollients and a follow-up at 2 months showed partial improvement. In view of the benefit of d-penicillamine therapy for his Wilson's disease and mild symptomatic skin lesions, the d-penicillamine therapy was continued.
To date, there has been no published case of d-penicillamine therapy-induced Grover's disease in the pediatric age group. Though clinically, it was difficult to diagnose Grover's disease in our case, both dermoscopy and histopathology aided the diagnosis. The Darier variant of Grover's disease is present with acantholysis similar to Darier's disease but its presence is very subtle and multiple visualizations are required. The dermoscopy also had typical features consistent with Grover's disease. Based on the literature review, clinicopathological and dermoscopy correlation, we arrived at the diagnosis of Grover's disease.
Our case highlights the unique feature of a persistent variant of Grover's disease with keratotic papules (Darier variant) and Koebnerization probably caused by direct toxicity of the d-penicillamine therapy.
Learning points
- Grover's disease, also called TAD, is rarely seen in children. The skin lesions can often be persistent and pruritic for a few months to years.
- The exact etiopathogenesis in children is not known.
- Clinically, it presents as papules, papulovesicles, pustules, bullae, erythematous plaque-like lesions, nodules, and rarely as keratotic papules.
- Histologically, Grover's disease can resemble Darier's-like pattern, spongiotic pattern, Hailey-Hailey-like pattern, pemphigus vulgaris, or pemphigus foliaceus-like pattern.
- Acantholysis in Grover's disease is very subtle and multiple visualizations are required in the histopathological examination.
- D-Pencillamine therapy can cause Grover's disease by causing acantholysis due to disruption of the disulfide bonds of cell adhesion molecules.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
| References | | |
| 1. | Bellinato F, Maurelli M, Gisondi P, Girolomoni G. Clinical features and treatments of transient acantholytic dermatosis (Grover's disease): A systematic review. J Dtsch Dermatol Ges 2020;18:826-33.  |
| 2. | Chalet M, Grover R, Ackerman AB. Transient acantholytic dermatosis: A reevaluation. Arch Dermatol 1977;113:431-5. |
| 3. | Weaver J, Bergfeld WF. Grover disease (transient acantholytic dermatosis). Arch Pathol Lab Med 2009;133:1490-4. |
| 4. | Gantz M, Butler D, Goldberg M, Ryu J, McCalmont T, Shinkai K, et al. Atypical features and systemic associations in extensive cases of Grover disease: A systematic review. J Am Acad Dermatol 2017;77:952-7. |
| 5. | Zvulunov A, Grunwald MH, Avinoach I, Halevy S. Transient acantholytic dermatosis (Grover's disease) in a patient with progressive systemic sclerosis treated with D-penicillamine. Int J Dermatol 1997;36:476-7. |
| 6. | Asahina A, Ishiko A, Saito I, Hasegawa K, Sawamura D, Nakano H. Grover's disease following multiple bilateral Blaschko lines: A rare clinical presentation with genetic and electron microscopic analyses. Dermatology 2012;225:183-7. |
| 7. | Errichetti E, De Francesco V, Pegolo E, Stinco G. Dermoscopy of Grover's disease: Variability according to histological subtype. J Dermatol 2016;43:937-9. |
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