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Table of Contents
Year : 2021  |  Volume : 39  |  Issue : 1  |  Page : 45-46

Mal de Meleda mimicking psoriasis: A case report and literature review

Department of Dermatology, Drug Hypersensitivity Clinical and Research Center, Chang Gung Memorial Hospital, Taipei, Linkou and Keelung; College of Medicine, Chang Gung University, Taoyuan, Taiwan

Date of Submission13-May-2020
Date of Decision12-Jun-2020
Date of Acceptance13-Jun-2020
Date of Web Publication26-Feb-2021

Correspondence Address:
Dr. Rosaline Chung-Yee Hui
Department of Dermatology, Chang Gung Memorial Hospital, Keelung Branch, No. 222, Maijin Rd., Anle Dist., Keelung City
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ds.ds_31_20

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How to cite this article:
Wang HN, Wang FY, Hui RC. Mal de Meleda mimicking psoriasis: A case report and literature review. Dermatol Sin 2021;39:45-6

How to cite this URL:
Wang HN, Wang FY, Hui RC. Mal de Meleda mimicking psoriasis: A case report and literature review. Dermatol Sin [serial online] 2021 [cited 2023 Mar 27];39:45-6. Available from: https://www.dermsinica.org/text.asp?2021/39/1/45/310238

Dear Editor,

A 58-year-old male patient presented with hyperkeratotic plaques over the palms and soles since he was 10-month-old. The patient denied consanguineous marriage in the family. His sister also presented with similar symptoms. Physical examination revealed erythematous scaly plaques on the palms and soles extending to the limbs, forming a “glove-and-socks” distribution [Figure 1]. There were thick erythematous plaques on the scalp, face, lower back, elbows, bilateral legs, and thighs as well. Dystrophic nails, periodic angular cheilitis, and alopecia were also noted, especially over the frontal hairline. However, the patient denied arthralgia, hyperhidrosis, problem of the eye, or tooth loss. Skin biopsy from the abdomen showed parakeratosis with Munro's microabscess, the absence of the granular layer, moderate acanthosis, exocytosis, papillomatosis with congested vessels, and perivascular lymphocytic infiltrate in the dermis. Due to the unique clinical presentation, we arranged DNA sequencing examination for him. Sanger sequencing revealed a previously reported homozygous c. 256G > A mutation in the SLURP-1 gene [Figure 2]. These findings confirmed the diagnosis of  Mal de Meleda More Details (MDM).{Figure 1}{Figure 2}

The patient had taken long-term acitretin for >20 years, in combination with methotrexate 15 mg/week, but periodic desquamation over skin lesions was still noted. Therefore, secukinumab 300 mg was given every 2 weeks in combination with acitretin; a partial improvement over the hairline plaques was noted, but erythematous plaques on the palms and soles persisted. The patient then received adalimumab 40 mg every other week, but the plaques still progressed to the face even after adalimumab treatment was started.

MDM is a rare autosomal recessive skin disorder characterized by transgradient palmoplantar keratoderma. MDM is caused by a mutation in the SLURP-1 gene encoding secreted lymphocyte antigen 6/urokinase-type plasminogen activator receptor-related protein-1 (SLURP-1).[1] SLURP-1 is a secreted protein that regulates epidermal differentiation. Defective SLURP-1 may lead to impaired T-cell activation,[2] the proliferation of keratinocytes[3], and the release of inflammatory cytokines such as tumor necrosis factor (TNF)-alpha. SLURP-1 can also inhibit the growth of epithelial cancer cells in vitro.[4] Several cases of Bowen disease, squamous cell carcinoma, and melanoma arising from the hyperkeratotic lesions of patients with MDM have been reported.[5],[6]

MDM is characterized by transgradient erythema over the palms and soles, and the skin lesions tend to exacerbate with age. MDM can also mimic psoriasis when it involves other body sites such as the scalp, elbows, and knees. The diagnosis of MDM is based on the inheritance pattern, age at onset, morphology, and distribution of hyperkeratosis, but the mutational analysis is the gold standard to confirm the diagnosis of MDM[7] and affects the following treatment.

MDM needs to be separated from psoriasis because of different treatment responses to biologic agents and the potential risk of cutaneous malignancies. The onset of the disease and family history provides some clues; MDM generally occurs soon after birth and has the autosomal recessive mode of inheritance. Both MDM and psoriasis can exhibit well-circumscribed red scaly plaques over other body sites, and both can have nail changes such as subungual hyperkeratosis and onycholysis. However, MDM is uniquely characterized by a stocking and glove pattern (transgradiens). Besides, sclerodactyly and digital constriction, as important signs of MDM, are uncommon in psoriasis. Histologically, it is difficult to differentiate between psoriasis and MDM; both can exhibit parakeratosis, orthokeratosis, acanthosis, Munro's microabscess, and perivascular lymphocytic infiltrate. However, SLURP-1 mRNA expression was upregulated by interleukin (IL)-22 in psoriasis[8], whereas SLURP-1 was absent or barely detectable in MDM, and hence, SLURP-1 immunostaining may, thus, help physicians to differentiate MDM from psoriasis. Finally, while the important cytokine network, including TNF-alpha, IL-17, and IL-22 is well-studied in psoriasis, its importance in MDM remains to be elucidated, and biologic agents targeting at TNF-alpha or IL17A had limited response in our patient. Systemic retinoids and topical keratolytic formulations have been used to treat MDM, but methotrexate and cyclosporine, as common systemic therapies for psoriasis, have a limited role in MDM.

In summary, dermatologists should differentiate MDM from psoriasis because they may share similar symptoms, but the response to treatments and disease prognoses are quite different. The extremely early age of onset, autosomal recessive inheritance, and the unique transgradient palmoplantar keratoderma should help differentiate the current case from the more common psoriasis vulgaris. Although TNF-alpha antagonists and IL-17A inhibitors were of limited use, systemic retinoids remain a plausible treatment option for patients with MDM.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Chimienti F, Hogg RC, Plantard L, Lehmann C, Brakch N, Fischer J, et al. Identification of SLURP-1 as an epidermal neuromodulator explains the clinical phenotype of Mal de Meleda. Hum Mol Genet 2003;12:3017-24.  Back to cited text no. 1
Tjiu JW, Lin PJ, Wu WH, Cheng YP, Chiu HC, Thong HY, et al. SLURP1 mutation-impaired T-cell activation in a family with mal de Meleda. Br J Dermatol 2011;164:47-53.  Back to cited text no. 2
Arredondo J, Chernyavsky AI, Webber RJ, Grando SA. Biological effects of SLURP-1 on human keratinocytes. J Invest Dermatol 2005;125:1236-41.  Back to cited text no. 3
Lyukmanova EN, Bychkov ML, Sharonov GV, Efremenko AV, Shulepko MA, Kulbatskii DS, et al. Human secreted proteins SLURP-1 and SLURP-2 control the growth of epithelial cancer cells via interactions with nicotinic acetylcholine receptors. Br J Pharmacol 2018;175:1973-86.  Back to cited text no. 4
Mozzillo N, Nunziata CA, Caracò C, Fazioli F, Botti G, Melanoma Cooperative Group. Malignant melanoma developing in an area of hereditary palmoplantar keratoderma (Mal de Meleda). J Surg Oncol 2003;84:229-33.  Back to cited text no. 5
Tourlaki A, Bentivogli M, Boneschi V, Brambilla L. Genetically proven Mal de Meleda complicated by Bowen's disease of the sole. Eur J Dermatol 2011;21:292-4.  Back to cited text no. 6
Guerra L, Castori M, Didona B, Castiglia D, Zambruno G. Hereditary palmoplantar keratodermas. Part II: syndromic palmoplantar keratodermas-Diagnostic algorithm and principles of therapy. J Eur Acad Dermatol Venereol 2018;32:899-925.  Back to cited text no. 7
Moriwaki Y, Takada K, Nagasaki T, Kubo N, Ishii T, Kose K, et al. IL-22/STAT3-Induced Increases in SLURP1 expression within psoriatic lesions exerts antimicrobial effects against Staphylococcus aureus. PLoS One 2015;10:e0140750.  Back to cited text no. 8


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