Extensive pyoderma gangrenosum-like lesions revealing a case of hyperzincemia and hypercalprotectinemia: when to suspect it?<sup>,</sup>

Resende, Ludimila Oliveira; Jorge, Marilia Formentini Scotton; Schmitt, Juliano Vilaverde

doi:10.1016/j.abd.2018.12.001

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Case Report • An. Bras. Dermatol. 94 (6) • 2019 • https://doi.org/10.1016/j.abd.2018.12.001 copy

Extensive pyoderma gangrenosum-like lesions revealing a case of hyperzincemia and hypercalprotectinemia: when to suspect it?^☆ ☆ How to cite this article: Resende LO, Jorge MFS, Schmitt JV. Extensive pyoderma gangrenosum-like lesions revealing a case of hyperzincemia and hypercalprotectinemia: when to suspect it? An Bras Dermatol. 2019;94:713-6. ^,^☆☆ ☆☆ Study conducted at the Hospital das Clínicas, Faculdade de Medicina de Botucatu, Universidade Estadual Paulista, Botucatu, SP, Brazil.

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Abstract

Hyperzincemia and hypercalprotectinemia is a rare inflammatory disease caused by a mutation in the PSTPIP1 gene, with a dysregulation of calprotectin metabolism. Calprotectin is a zinc-binding protein with antimicrobial properties and pro-inflammatory action. The authors report the case of a 20 year-old girl with cutaneous ulcers comparable with pyoderma gangrenosum, growth failure and chronic anemia, who was given the diagnosis of hyperzincemia and hypercalprotectinemia. Measurement of serum zinc and calprotectin concentrations are indicated in these cases.

KEYWORDS
Arthritis; Inflammation; Skin manifestations; Zinc

	Previous reports								Current report
Age at diagnosis	18	18	9	14	35	21	10	5	20
Sex	M	M	F	M	F	M	F	M	F
Growth	Reduced	Reduced	Reduced	Reduced	Normal	Normal	Reduced	Reduced	Reduced
Hepatosplenomegaly	-	Yes	Yes	Yes	Yes	Yes	Yes	Yes	Yes
Skin lesions	PG	Vasc	No	No	Vasc, eczema	Vasc, ulcers	PG	Eyelid lesions	PG
Rheumatologic changes	-	Arthritis	Arthritis	Arthritis	Arthritis, uveitis	Arthritis	Arthritis	Arthritis	Arthritis
CRP (<10 mg/L)	-	41-143	100-200	22	17	45-146	60	108	35
Hemoglobin (g/L)	-	8	9	10.9	12.5	8	5.5	7.6	7.3
Zinc (10-18 µm/L)	120-197	180-200	82-96	160-200	175	77	183	152	138
Calprotectin (<0.001 g/L)	-	6.5	1.4-2.55	9	6.1	1.5	12.5	2.3	0.6

Feature	Value
Age at diagnosis (years) ^a a Mean (standard deviation).	18.1 (8.7)
Sex
Female	3/7 (43%)
Male	4/7 (57%)
Hepatosplenomegaly	6/6 (100%)
Growth deficit	5/7 (71%)
Arthritis	6/6 (100%)
Anemia	5/6 (83%)
Hemoglobin (g/L) ^a a Mean (standard deviation).	8.2 (2.1)
Serum zinc (µm/L) ^a a Mean (standard deviation).	153.4 (35.4)
Calprotectin (g/L) ^b b Median (1st quartile - 3rd quartile).	4.9 (1.5-6.1)

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[1] * Corresponding author. E-mail:ludi.lor07@gmail.com (L.O. Resende).